S e c t i o n
3
Short Cases
22 Non-thyroid Neck Swelling
Case
Examination of non-thyroid neck swellings:
Diagnostic algorithm for a neck swelling
1. Identify the anatomical situation of the swelling (in relation to the triangle in the neck)
↓
2. Decide the plane of the swelling
↓
3. Recollect your anatomy (what are the normal anatomical structures situated in the region of the swelling in that plane)
↓
4. Check for mobility/fixity of the swelling
↓
5. Find out the external (size, shape, surface, edge, temperature, tenderness, etc.) and internal features of the
lump (solid or cystic, compressible/ reducible, pulsation, transillumination) and auscultation of the swelling
↓
6. Find out its effect on the surrounding tissue (feel the superficial temporal artery, examine the relevant cranial
nerves and look for Horner’s syndrome)
↓
7. Look for regional nodes (if the swelling is a node look for another group and contralateral side of the neck)
↓
8. In the case of paired organs like salivary gland, look for contralateral pathology also
↓
9. Look for a primary lesion (scalp, oral cavity, pharynx, hidden areas, etc.)
↓
10. Come to an anatomical diagnosis
↓
11. Come to a pathological diagnosis (Decide whether it is congenital /traumatic/inflammatory/neoplastic—
primary or secondary)
↓
12. If it is an organ concerned with function decide whether it is hyper functioning, normally functioning or hypofunctioning (functional diagnosis). The final diagnosis = Anatomical + Pathological + Functional diagnosis.
286
Clinical Surgery Pearls
Q 1. What are the causes for non-thyroid neck
masses?
All regions
• Skin and subcutaneous tissue
1. Sebaceous cyst
2. Lipoma
3. Neurofibroma
• Lymphadenopathy
1. Acute infection
2. Chronic infection—tuberculosis
3. Primary malignant—lymphoma
4. Secondary malignant—metastasis
Midline
1. Sublingual dermoid
2. Thyroglossal cyst/fistula
3. Pharyngocele
4. Laryngocele.
Note: 3 and 4 do not lie in the midline but arise
from the midline.
Lateral
1. Parotid swellings
2. Submandibular salivary gland
3. Branchial cyst
4. Carotid body tumor
5. Carotid aneurysm
6. Carotid tortuosity
7. Cystic hygroma
8. Subclavian aneurysm.
Triangles of the Neck
The neck is divided into Anterior and Posterior
triangles (Fig. 22.1).
The boundaries of the anterior triangle are:
• Midline
• Anteriorborderofthesternomastoid(oncologically
the boundary is the posterior border of the
sternomastoid)
• Inferior border of the ramus of the mandible.
The anterior triangle is further divided into:
• Digastric
• Carotid
• Muscular.
The boundaries of the posterior triangle are:
• Posterior border of the sternomastoid
• Anterior border of trapezius
• Upper border of the middle-third of the
clavicle.
Table 22.1 showing the Triangles of the neck, its
boundaries, contents and possible swellings in each
area.
Fig. 22.1: Triangles of the neck
Non-thyroid Neck Swelling
287
Table 22.1: Triangles of the neck
Triangle Boundaries Contents Possible swellings
Digastric • Inferior border of the ramus of
the mandible
• Anterior portion of the digastric
muscle
• Posterior portion of the digastric
muscle
• Midline
• Submandibular gland
• Lymph node
• Facial artery
• Submandibular swellings—sialadenitis, tumor
• Lymph nodes swelling
• Ranula (plunging)
• Sublingual dermoid
Carotid • Anterior belly of omohyoid
• Anterior border ofsternomastoid
• Posterior belly of digastric
• Commoncarotidarterydividingto
internal and external at the level of
hyoid bone
• Vagus nerve
• Lymph nodes
• Internal jugular vein
• Carotid body tumor
• Branchial cyst
• Carotid aneurysm
• Pharyngocele
Muscular • Anterior belly of digastric
• Anterior belly of omohyoid
• Midline
• Thyroid—may extend beyond
this area including the posterior
triangle
• Laryngeal structures
• Thyroid swelling
• Laryngocele
• Innominate aneurysm
Posterior • Posterior border ofsternomastoid
• Anterior border of trapezius
• Upper border of middle third of
clavicle
• Lymph nodes
• Accessory nerve
• Scalenus anterior muscle
• Thyroid swelling
• Cystic hygroma
• Lymph nodes
• Subclavian aneurysm
*Note: Skin and subcutaneous swellings like lipoma, sebaceous cyst and neurofibroma can occur in all the regions.
Remember the etiology of non-thyroid neck mass. Midline swellings of the neck
1. Sublingual dermoid
2. Plunging ranula
3. Thyroglossal cyst
4. Pharyngocele
5. Laryngocele
6. Swellings from isthmus of thyroid
7. Prelaryngeal lymph node
8. Pretracheal lymph node
9. Lymph nodes in the space of burns.
Remember the hidden areas of primary for a
metastasis in the neck.
Hidden areas for primary
• Pyriform sinus • Base of tongue
• Vallecula • Fossa of Rosenmüller
• Tonsillar fossa
Remember the midline swellings of the neck.
They are from above downwards:
23 Tuberculous Cervical
Lymph Node
Case
Case Capsule
A 20-year-old male patient with multiple swellings
on the side of the neck involving the jugulodigastric group of lymph nodes and nodes on the
anterior and posterior triangles of neck. The nodes
are of varying consistency, some are soft and some
are firm. The jugulodigastric and upper deep
cervical nodes are matted together. The patient
complains of evening rise of temperature.
Read the diagnostic algorithm for a neck swelling.
Read the checklist of case no. 15 of long cases
Checklist for history
• Family history of tuberculosis
• H/o exposure to tuberculosis
• H/o BCG vaccination
• H/o evening rise of temperature
• H/o loss of appetite and loss of weight
Checklist for lymph node examination:
1. Remember the pneumonic – PALS (P – Look for
Primary lesion in the drainage area, A – Look for
Another lymph node, L – Look for Liver, S – Look
for Spleen) (Read case no.15 of long cases)
Tuberculous Cervical Lymph Node
289
2. Remember the 3 lymphatic water sheds in the
body forthe skin lymphatic drainage (Read case
no.15 of long cases).
3. Remember the order of palpation of cervical
lymph nodes (Fig. 23.1)
4. Remember the causes for matting of lymph
nodes
5. Always examine the oral cavity including the
tonsil
6. Examine the chest for evidence of pulmonary
tuberculosis.
Q 1. What is the anatomical diagnosis in this case?
Lymph nodes.
Q 2. What are the diagnostic points in favor of
lymph nodes?
1. Shape of the swelling
2. Plane of the swelling—deep to deep fascia.
Q 3. What is the plane of the cervical lymph nodes?
For all practical purposes majority of the cervical
Fig. 23.1: Cervical lymph node examination lymph nodes are deep to deep fascia.
Flow chart 23.1: Classification of cervical lymph nodes
290
Clinical Surgery Pearls
Now proceed upwards to the external jugular
nodes (superficial).
Q 9. What are the causes for lymphadenopathy?
The causes may be classified as shown in Flow
chart 23.2.
Q 10. What is the plan of action for finding out the
source of the enlarged lymph nodes?
Search for primary lesion in cervical lymphadenopathy.
Start from above and work downwards
1. Examine the skin of the scalp, face, ears and neck
2. Examine the nose
3. Transilluminate the air sinuses
4. Examine the oral cavity
5. Examine the nasopharynx and larynx (ENT
examination)
6. Palpate the salivary glands (parotid and submandibular)
7. Examine the thyroid gland
8. Examine the breast
9. Examine the chest
10. Examine the abdomen and genitalia.
Q 11. What is the pathological diagnosis in this
case?
Most probably thisis a case of tuberculous cervical
lymphadenitis.
Q12. What are the points in favor of tuberculosis
of the lymph node?
1. Matting of lymph nodes
2. Varying consistency of the nodes
3. Jugulodigastric node is affected (which is the
most common group affected in tuberculosis)
4. Evening rise of temperature.
Q 4. What are the groups of lymph nodes which are
superficial to the deep fascia in the neck?
1. The external jugular group of lymph nodes
2. The submental nodes
3. The occipital nodes
4. The facial nodes
5. Postauricular nodes.
Q 5. How many lymph nodes are there in the neck?
• Roughly 300 nodes (out of total 800 nodes in
the human body)
• About 150 are in the mesentery.
Q 6. What are the functions of lymph nodes?
1. Filtration of effete cells, bacteria and antigens
2. Presentation of antigens to the lymphocytes
3. Regulation of protein content of efferent lymph.
Q 7. How will you classify cervical lymph nodes?
They may be classified asshown in flow chart 23.1.
Note: SM muscle—sternomastoid muscle.
Q 8. What is the sequence of palpation of cervical
lymph nodes?
The following sequence is recommended. Start
palpitating from above from the submental node
and proceed backwards (Fig. 23.1).
1. Submental
2. Submandibular
3. Preauricular
4. Postauricular
5. Occipital.
Then proceed downwards laterally from the
jugulodigastric
6. Jugulodigastric
7. Deep cervical
8. Jugulo-omohyoid
9. Scalene
10. Supraclavicular.
Tuberculous Cervical Lymph Node
291
Q 13. What are the causes for matting?
Causes for matting of lymph nodes:
1. Tuberculous lymphadenitis
2. Acute lymphadenitis
3. Late stages of lymphoma
4. Late stages of metastasis neck.
Q 14. What is the cause for matting?
The organism will reach the lymph node from the
primary focus via the lymphatics. The lymphatics
are distributed along the periphery of the lymph
nodes and from there it will reach the subcapsular
sinus. The subcapsular sinuses of adjacent lymph
nodes are involved subsequently and this will
produce matting.
Q 15. Can you get tuberculosis of the nodes
without matting?
Yes. In miliary tuberculosis there won’t be any
matting. The organisms coming via the blood
vessels enter the medullary region directly and
therefore there is no periadenitis and matting in
miliary tuberculosis.
Q 16. What is the anatomy of the cut section of
lymph node?
The lymph node is kidney-shaped and it has got a
hilum. The afferent and efferent vessels enter and
leave the hilum. It has got a capsuleandbeneaththe
capsule there is the subcapsular space. All around
the lymph nodes you get the lymphatics reaching
Flow Chart 23.2: Classification of causes for lymphadenopathy
292
Clinical Surgery Pearls
the capsule and sub-capsular space. Organisms
coming to the lymph node thus reaches the
subcapsularspace initially.The lymphnodehasgot a
cortex and a medulla.The cortexhaslymph follicles
which issituated externally. Beneath the cortex you
get the medulla where the medullary cords are seen.
Organisms coming via the vessels reach directly the
medulla in contrast to the lymphatics(Fig. 23.2).
Q 17. What is the primary focus for cervical lymph
node tuberculosis?
The tonsil is usually the primary focus for the
cervical node tuberculosis. This can lead to
jugulodigastric node enlargement (the tonsillar
group of lymph nodes) and from there it will reach
other groups in the neck.
Q 18. If you get isolated posterior triangular group
of nodes which are proved to be tuberculosis,
what is the likely primary focus?
Adenoids.
Q 19. What is the incidence of cervical node tuberculosis secondary to pulmonary tuberculosis?
• In 80% of cases the tuberculosis process is
limited to the affected lymph nodes
• Primary focus in the lungs must always be
suspected and investigated
• Atypical mycobacterial adenitis is seldom
associated with pulmonary tuberculosis.
Q 20. What are the groups of lymph nodes affected
by tuberculosis in the body?
1. Upper deep cervical
2. Supraclavicular
3. Mediastinal nodes
4. Axillary
5. Inguinal nodes.
Note:
a. Supraclavicular nodes represent the upward
extension of hilar and mediastinal lymphadenopathy
b. Axillary and inguinal nodes are involved by
hematogenous or retrograde lymphatic spread.
Q 21. What are the pathological stages of
tuberculous lymph nodes?
There are five stages (Fig. 23.3):
Stage 1: The lymph nodes are enlarged and
solid. There is no matting of lymph nodes (no
periadenitis).
Stage 2: The lymph nodes are large, firm and
matted together (fixed to each other) because of
the periadenitis.
Stage 3: Stage of caseation and cold abscess—
The lymph nodes breakdown, and liquefy. The pus
will collect beneath the deep fascia. A fluctuant
mass will be palpated without any overlying skin
Fig. 23.2: Lymph node section inflammation (cold abscess). In addition, nodes
Tuberculous Cervical Lymph Node
293
without softening will also be present which will
give rise to varying consistency.
Stage 4: Stage of collar-stud abscess—The deep
cervical fascia is eroded eventually resulting in the
escape of pus beneath the superficial fascia which
is a laborious space.
Stage 5: Stage of sinuses and ulcers—The pus
will eventually burst through the skin resulting in
a discharging sinus or ulcer.
Q 22. Will the cold abscess contain tubercle bacilli?
Yes. The abscess is lined by granulation tissue and
caseous material.
Q 23. How a tuberculous ulcer is formed?
Once a sinus is formed the discharge will infect the
surrounding skin and cause ulceration.
Q 24. What are the characteristics of tuberculous
ulcer?
The tuberculous ulcer has got undermined edges,
seropurulent discharge and pale granulation tissue.
Q 25. What is collar-stud abscess?
Thepusisformedasa resultofbreakdownofa lymph
node which willsubsequently erode the deep fascia
andcome tothe spacebeneaththe superficialfascia.
There is a superficial soft swelling, which is nothing
but pus, and is communicating with the offending
lymph node situated deep to the deep fascia. The
node, the connection and the superficial soft
swelling together will take the shape of a collar-stud.
Q 26. What are the clinical types of tuberculous
lymphadenitis?
There are four types:
Fig. 23.3: Tuberculosis lymphadenitis stages in order
294
Clinical Surgery Pearls
1. Acute type
– Seen in infants and children below five years
– Inflammatory signs may be there and may
resemble acute lymphadenitis.
2. Caseating type—commonest type seen in
young adults with typical matted nodes with
caseation, cold abscess and sinuses.
3. Hyperplastic type—this is seen in patients with
good resistance. The lymphoid hyperplasia is
more predominant than caseation. The nodes
are usually firm and discrete.
4. Atrophic type—seen in elderly where the lymph
nodes undergo natural involution. The nodes are
small and burst resulting in caseation.
Q 27. What is scrofuloderma?
The skin involvement as a result of tuberculosis is
called scrofuloderma. The skin will be discolored
bluish or hyperpigmented.
Q 28. How will you investigate and confirm your
diagnosis?
1. ESR—will be raised
2. X-raychest—evidenceofpulmonarytuberculosis
need not be there because majority of the
cervical lymph node tuberculosis are primary
(and not secondary to pulmonary tuberculosis)
3. Tuberculintest(Mantoux)—apositive testhasno
diagnostic value (because of BCG vaccination) a
negative test is useful in excluding tuberculosis
4. FNAC of the lymph node
5. Biopsy of the lymph node for pathology and
microbiology
6. Aspiration of the cold abscess for AFB staining.
Q 29. How Mantoux test is done?
One unit of PPD (Purified Protein Derivative) is
injected intradermally in the volar surface of the
forearm. After 48 hours, look for area of induration
surrounding the injection site. A positive test is
one where the indurationexceeds12mm. A positive
test is suggestive of prior or present infection with
M. tuberculosis. Negative results do not always rule
out tuberculosis (immunosuppression, malnutrition
and diseases like lymphomas suppress the test).
Q 30. Is FNAC of the node reliable?
For lymph node pathology biopsy of the intact
lymphnodeisalwayssuperiorbecauseapathologist
can study the architecture of the lymph node (this is
not possible with FNAC). Ifthe needle isstriking the
granuloma in the lymph node, the pathologist will
give a positive report. Therefore, biopsy is always
a superior investigation for lymph node.
Q 31. What are the important points to be remembered while taking cervical lymph nodes for biopsy?
1. Always try to take an intact node for biopsy
2. If possible take 2 nodes (1 node for the
pathologist and 1 node for the microbiologist)
3. Send the lymph node for the pathologist in formalin
4. Send the lymph node for the microbiologist in
saline solution (this is for AFB culture).
Q 32. What is the media used for culture and how
long it will take to get a positive culture?
• Lowenstein—Jensen media
• Takes 6 weeks for positive culture
• Selinite medium—shortens the time of growth
to 5 days.
Q 33. What are the new methods for the diagnosis
of tuberculosis?
PCR – The result will be ready in one week.
BACTECT – (Using radioactive C14) Result will be
ready in 4 days
Q 34. Suppose there is only one lymph node,
would you attempt FNAC?
No. FNAC will induce changes in the lymph node,
which are likely to alter the pathological picture if it
Tuberculous Cervical Lymph Node
295
issubsequently taken for biopsy study.Thisis called
WARF (Worrisome Anomaly Related to FNAC).
Q 35. What are the pathological changes in the
lymph node?
Pathologically we get the tubercle, which consists
of an area of caseation surrounded by giant cells,
epithelioid cells, lymphocytes and plasma cells.
Q 36. What is the difference between caseous
material and tuberculous pus?
Caseous material—dry, granular and cheese
like material is called caseous material (granular
structure less material).
Tuberculous pus—softening and liquefaction of
the caseous material results in the formation of a
thick creamy fluid called tuberculous pus. Itis highly
infective because liquefaction is associated with
multiplication of the organism. In addition, it contains
fatty debrisin serousfluid with few necrotic cells.
Q 37. Can you start antituberculous treatment
empirically without pathological or microbiological
report?
No. Pathological or microbiological support is
necessary forstarting the antituberculoustreatment.
Q 38. What are the organisms responsible for
lymph node tuberculosis?
• Human tuberculosis
• Bovine tuberculosis.
Q 39. What are the organisms responsible for
atypical Mycobacterium tuberculosis?
• Mycobacterium avium intracellulare
• Mycobacterium scrofulaceum.
Q 40. Is there any role for urine examination?
Yes. The renal and pulmonary tuberculosis
occasionally coexist. Therefore, the urine should
be carefully examined.
Q 41. Is it possible to get calcified cervical lymph
node without prior symptoms of cervical lymph
node tuberculosis?
Yes. In patients with natural resistance to the
infection, the nodes may accidentally detected at
a later date as calcified nodes.
Q 42. What is the treatment of choice in a proved
case of cervical lymph node tuberculosis?
Antituberculous chemotherapy – Triple Drug
Therapy– Rifampicin, INH and Ethambutol—(Read
the chapter on long cases- right iliac fossa mass -
abdominal tuberculosis).
Q 43. What are the indications for surgery in
cervical lymph node tuberculosis?
Indicationsforsurgery in tuberculouslymph-adenitis
1. Biopsy (the most important indications)
2. Excisionofagroupofnodesifitisnot responding
to antituberculous treatment
3. Excision of the offending lymph node in cases
of collar-stud abscess
4. Excision of abscess if it is not responding to
aspiration
5. Persistent sinus.
Q 44. What are the causes for persistent sinus?
Causes for persistent sinus:
1. Secondary infection
2. Considerable fibrosis
3. Necrotic and calcified material replacing the
lymph node.
Q 45. If the cervical nodes are not responding to the
antituberculous drugs what should be suspected?
Atypical mycobacterial adenitis.
Q 46. What is the treatment of cold abscess?
Aspiration. Incision and drainage is not recommended
because it willresultin sinusformation.
Q 47. How will you aspirate cold abscess?
Aspiration is done through the nondependent part.
24 Cervical Metastatic Lymph Node
and Neck Dissections
Case
Case Capsule
A 65-year-old male patient presents with a hard
lymph node swelling of 3 cm size involving the level
III group on right side. The swelling is mobile. The
superficial temporal artery is palpable. The cranial
nerves are normal. There are no abdominal, chest or
ENT complaints. The patient is apparently healthy.
Read the diagnostic algorithm for a neck swelling.
Checklist for history
1. Alcohol and tobacco use in history
2. Pain around the eyes – referred from the nasopharynx
3. Otalgia—carcinoma base of tongue, tonsil, and
hypopharynx can cause otalgia
4. Odynophagia—as a result of cancers of the
base of the tongue, hypopharynx, cervical node
metastasis, etc.
5. Bleeding from the nose (epistaxis)—cancers of the
nasal cavity
6. Hemoptysis
7. Alteration of phonation
8. Difficulty in breathing
9. Difficulty in swallowing—late symptom of base of
tongue, hypopharynx and cervical esophagus
10. Difficulty in hearing—from nasopharynx
11. Hoarseness of voice—carcinoma glottis and
carcinoma thyroid
12. History of prior SCC.
Checklist for examination
1. Careful examination of oral cavity after removal of
dentures
2. Bimanual palpation of the floor of the mouth
3. Check for nasal block
4. Check for sensory loss in the distribution of
infraorbital nerves—maxillary sinus cancer
5. Examine the cranial nerves III–VII and IX–XII
(involvement in nasopharyngeal cancer)
6. Look for Horner’s syndrome—involvement of
cervical sympathetic chain, extralaryngeal spread
of laryngeal cancer and extracapsular invasion of
cervical lymph node
7. Look for trismus
8. A thorough ENT examination
9. Examination of thyroid
10. Examination of salivary glands
11. Examination of breast
12. Examination of chest
13. Examination of abdomen. Contd...
Contd...
Cervical Metastatic Lymph Node and Neck Dissections
297
Checklist for evaluation of metastatic
cervical lymph nodes
1. Clinical examination of ipsilateral and contralateral
neck.
2. Palpation of thyroid gland and parotid gland
3. Examination of oral cavity
4. Examine the tonsillar region
5. Laryngoscopy (both direct and indirect)
6. Examination of nasopharynx
7. Examination of hypopharynx
Q 5. What are the other clinical examinations?
1. Examination of breast for a primary lesion
2. Examination of chest for a primary lesion
3. Examination of abdomen for visceral malignancy.
Q 6. If all these clinical examinations are negative
what is the course of action?
An examination under anesthesia (EUA)—followed
by Panendoscopy.
Q 1. What is the most probable diagnosis in this
case?
Metastatic lymph node.
Q 2. Why metastatic lymph node?
• Since the lymph nodes are hard, one should
suspect a malignant node
• It is a disease of old age (mean age for male is
65 years and female 55 years)
• Males are more affected than females (4:1)
• 85%ofthe malignant nodes are metastatic (only
15% are primary)
• 85% are likely to have a primary in the
supraclavicular region.
Q 3. What is the most important clinical
examination in such a patient?
A complete head and neck examination is required
(since 85% are having a supraclavicular primary).
Q 4. What are the areas to be examined in the
head and neck?
298
Clinical Surgery Pearls
Q12. What is the order of frequency of primary in
a case of metastasis?
• Head and neck source of primary: The primary
sites in order of frequency are:
1. Nasopharynx
2. Tonsil
3. Base of tongue
4. Thyroid
5. Supraglottic larynx
6. Floor of mouth
7. Palate
8. Pyriform fossa
• Nonhead and neck source of primary (in order
of frequency)
1. Bronchus
2. Esophageus
3. Breast
4. Stomach
Q 13. What is the contraindication for a preliminary
lymph node biopsy in a metastatic lymph node? (PG)
• Abiopsywill produce scarring of subcutaneous
tissue and will destroy the tissue planes. This
will affect the neck dissection if it becomes
necessary because the scar tissue can not be
distinguished from the tumor
• Biopsy will destroy nodal or fascial barriers
holding the cancer in check and seedling of the
soft tissues and lymphatics will occur
• Chancesforneck recurrence will occur as a result
of biopsy (recurrence is the major cause of death
rather than metastasis in SCC)
• Chances for general spread is high.
Q 14. If nothing is found after pan endoscopy and
blind biopsy, what next?
MRI of the neck is done.
Panendoscopy
• Nasopharyngoscopy
• Esophagobronchoscopy
• Laryngoscopy (direct).
Q 7. What is the purpose of esophagoscopy and
bronchoscopy?
In metastatic squamous cell carcinoma (SCC), 10-
20% chance for a second primary is there in the
aerodigestive tract.
Q 8. What is the definition of a “new primary” after
treatment of previous cancer?
One arising more than 3 years after previous cancer
is considered a new primary.
Q 9. If nothing is found on panendoscopy, what
next?
Surveillance biopsy: blind biopsies are taken from
the following areas.
Areas for blind biopsy
• Tonsils
• Tonsillar beds
• Base of tongue (posterior 1/3rd)
• Pyriform sinus
• Subglottic region
• Fossa of Rosenmüller
• Adenoids
• Retromolar trigone.
Q 10. If surveillance biopsy is negative how to
proceed?
Ipsilateral tonsillectomy.
Q 11. What is the purpose of surveillance biopsy?
In the absence of gross lesion, in 10–15% of cases
primary will be revealed by surveillance biopsy.
Cervical Metastatic Lymph Node and Neck Dissections
299
Q 15. Why MRI is superior to CT for evaluation of
a metastatic node of unknown primary?
• MRI can identify subtle changes in soft tissues
• Guided biopsy of the primary lesion is possible
• Extension ofthe primary to the surrounding soft
tissues can be identified.
Q 16. If MRI is negative, what is the next step?
FNAC.
Q 17. If FNAC is negative, what is the next step?
An open biopsy is indicated now. If metastatic SCC is
found on frozen section, it is immediately followed
by a neck dissection if it is operable.
Q 18. Why not a delayed neck dissection?
The best chance for cure and time for dissection is
when the normal tissue planes are intact. Thus, the
time to carry out a biopsy is when you are ready
to carry out a dissection.
Q 19. What are the possible FNAC or biopsy
reports?
Histological types of metastasis (50% SCC, 25%
poorly differentiated and 25% adenocarcinoma).
Histological type of metastasis
1. Squamous cell carcinoma (SCC)
2. Nonsquamous cell carcinoma
• Adenocarcinoma
• Poorly differentiated carcinoma
• Poorly differentiated neoplasm.
Q 20. If the report is adenocarcinoma what are
the possibilities?
Primary source for adenocarcinomatous deposits
in the neck nodes:
• Salivary neoplasm
• Thyroid carcinoma
• Breast carcinoma
• Occult lung cancer
• Prostatic cancer
• Renal malignancy
• GI malignancy.
Q 21. What is the treatment of metastatic
adenocarcinoma? (Flow chart 24.1)
There is no role for surgery because it is a
disseminated malignancy. Patient will go in for
chemotherapy (Paclitaxel and carboplatin).
Q 22. What is the management of poorly
differentiated neoplasm? (Flow chart 24.1) (PG)
Repeat the FNAC. If this too turns out to be
inconclusive, do a biopsy. If biopsy too proves to be
inconclusive do immunohistochemistry.
Q 23. What is the purpose of immunohistochemistry?
Immunohistochemistry and electron microscopy
is done to identify the lymphomas and other
chemoresponsive neoplasms (about 60%).
Q 24. What is the management of poorly differentiated carcinoma? (Flow chart 24.1) (PG)
Again immunohistochemistry and electron
microscopy are recommended in order to identify
the chemoresponsive subgroups:
• Lymphoma
• Ewing’s tumor
• Neuroendocrine tumors
• Primitive sarcomas.
Q 25. What is the commonest pathological type
of neck node metastasis?
Squamous cell carcinoma—80%.
300
Clinical Surgery Pearls
Q 26. What are the squamous cell carcinomas
which will metastasize bilaterally? (PG)
SCC with bilateral metastasis
1. Lower lip
2. Supraglottis
3. Soft palate.
Q 27. Which group of lymph node is involved in
carcinoma nasopharynx? (PG)
Nodes involved in carcinoma nasopharynx
• Retropharyngeal nodes
• Parapharyngeal nodes
• Level II – V.
Q 28. What are the carcinomas which will metastasize to retropharyngeal lymph nodes? (PG)
Malignancies involving the retropharyngeal nodes
1. Nasopharynx
2. Soft palate
3. Posterior and lateral oropharynx
4. Hypopharynx.
Q 29. What are the primary sites below the
clavicle?
Sites of the primary below the clavicle (15%)
• Lung (commonest)
• Pancreas
Flow chart 24.1: Management of occult primary
Contd...
Cervical Metastatic Lymph Node and Neck Dissections
301
• Esophagus
• Stomach
• Breast
• Ovary
• Testis
• Prostate.
Q 30. Which group of lymph nodes are involved
in infraclavicular primary?
The level IV and V (lower jugular chain and
supraclavicular nodes).
Q 31. What are the other investigations
recommended?
• X-ray chest
• Sputum cytology
• CT scan of the chest and abdomen
• Mammography
• PET scan (if required).
Q 32. What is the role of PET scan?
The 18-Fluorodeoxyglucose (18FDG) analog is
preferentially absorbed by neoplastic cells and can
be detected by positron emission tomography (PET)
scanning. It is more sensitive than CT in identifying
the primary lesion. But in the case of unknown
primary the sensitivity is not more than 50%. This
is because the unknown primary tumor may have
spontaneously involuted.
Q 33. What is the definition of occult primary?
When the lymph node is found to contain metastatic
carcinoma but the primary is unknown, even after
all these investigations, then it is called occult
primary.
Q 34. What are the levels of lymph nodes?
There are VII levels of lymph nodes
Level - I : Submental, submandibular
Level - II : Upper jugular
Level - III : Mid jugular
Level - IV : Lower jugular
Level - V : Posterior triangle (spinal accessory
and transverse cervical) (upper,
middle, and lower, corresponding
to the levels that define upper,
middle, and lower jugular nodes)
Level - VI : Prelaryngeal (Delphian), pretracheal, paratracheal
Level - VII : Upper mediastinal
Other groups: Suboccipital, retropharyngeal,
parapharyngeal, buccinator (facial),
preauricular, periparotid and
intraparotid.
Q 35. What are the boundaries of each level?
The boundaries are as follows (Fig. 24.1):
Level - I : It is bounded by the anterior and
posterior bellies of the digastric muscle
Fig. 24.1: Lymph node levels of neck
Contd...
302
Clinical Surgery Pearls
and the hyoid bone inferiorly and the
body of the mandibles superiorly
Level - II : Contains the upper jugular lymph
nodes and extends from the level of
the skull base superiorly to the hyoid
bone inferiorly (the nodes in relation to
the upper third of the internal jugular
vein – upper jugular group).
Level - III : Contains the middle jugular lymph nodes
from the hyoid bone superiorly to the
level of the lower border of the cricoid
cartilage inferiorly (nodes in relation to
the middle third of the internal jugular
vein – middle jugular group)
Level - IV : Contain the lower jugular lymph nodes
from the level of the cricoid cartilage
superiorly to the clavicle inferiorly
(nodes in relation to the lower third
of the internal jugular vein – lower
jugular group)
Level - V : Contains the lymph nodes in the
posterior triangle bounded by the
anterior border of the trapezius muscle
posteriorly, the posterior border of
the sternocleidomastoid muscle
anteriorly, and the clavicle inferiorly.
For descriptive purposes, Level V may be
further subdivided into upper, middle,
and lower levels corresponding to the
superior and inferior planes that define
Levels II, III, and IV.
Level - VI : Contains the lymph nodes of the anterior
central compartment from the hyoid
bone superiorly to the suprasternal
notch inferiorly. On each side, the lateral
boundary is formed by the medial
border of the carotid sheath.
Level - VII: Contains the lymph nodes inferior to
the suprasternal notch in the superior
mediastinum.
Note: Further divisions as per AJCC 7th edition
Level Superior Inferior Anterior (medial) Posterior (lateral)
IA Symphysis of
mandible
Body of hyoid Anterior belly of contra
lateral digastric muscle
Anterior belly of
ipsilateral digastric
muscle
IB Body of mandible Posterior belly of digastric
muscle
Anterior belly of digastric
muscle
Stylohyoid muscle
IIA Skull base Horizontal plane defined
by the inferior border of the
hyoid bone
The stylohyoid muscle Vertical plane
defined by the
spinal accessory
nerve
IIB Skull base Horizontal plane defined
by the inferior body of the
hyoid bone
Vertical plane defined by
the spinal accessory nerve
Lateral border of the
sternocleidomastoid
muscle
Contd...
Cervical Metastatic Lymph Node and Neck Dissections
303
VA Apex of the
convergence of the
sternocleidomastoid
and trapezius muscles
Horizontal plane defined
by the lower border of the
cricoid cartilage
Posterior border of the
sternocleidomastoid muscle
or sensory branches of
cervical plexus
Anterior border of
the trapezius muscle
VB Horizontal plane
defined by the lower
border of the cricoid
cartilage
Clavicle Posterior border of the
sternocleidomastoid muscle
Anterior border of
the trapezius muscle
Q 36. What are the probable primary sites for each
level? (PG)
Primary sites for each level of cervical lymph nodes
Lymph node level Primary cancer sites
Level I Oral cavity, lip, salivary gland,
skin
Level II Oral cavity, nasopharynx,
oropharynx, larynx, salivary
gland
Level III Oral cavity, oropharynx, hypopharynx, larynx, thyroid
Level IV Oropharynx, hypopharynx,
lar ynx, thyroid, cer vical
esophagus
Level V N a s o p h a r y n x , s c a l p
(Accessory nodes)
Level V G I tract, breast, lung
(supraclavicular)
Q 37. What is the area of drainage of suboccipital
nodes?
Skin of the scalp.
Q 38. What is the drainage area of parotid nodes?
Parotid gland and skin.
Q 39. What is the N (regional lymph node) staging?
N staging as per AJCC 7th edition
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
*N1 Metastasis in a single ipsilateral lymph node, 3
cm or less in greatest dimension
*N2 Metastasis in a single ipsilateral lymph node,
more than 3 cm but not more than 6 cm in
greatest dimension; or in multiple ipsilateral
lymph nodes, none more than 6 cm in greatest
dimension; or in bilateral or contralateral
lymph nodes, none more than 6cm in greatest
dimension.
*N2a Metastasis in single ipsilateral lymph node
more than 3 cm but not more than 6 cm in
greatest dimension
*N2b Metastasis in multiple ipsilateral lymph nodes,
none more than 6 cm in greatest dimension.
*N2c Metastasis in bilateral or contralateral lymph
nodes, none more than 6 cm in greatest
dimension
*N3 Metastasis in a lymph node more than 6cm in
greatest dimension
* Note: For Nasopharynx
N1 is unilateral metastasis in cervical lymph
node (s), 6 cm or less in greatest dimension, above
the supraclavicular fossa, and or unilateral or
bilateral retropharyngeal lymph nodes 6 cm or less
in greatest dimension.
Contd...
304
Clinical Surgery Pearls
N2 – Bilateral metastasis in cervical lymph node
(s), 6 cm or less in greatest dimension, above the
supraclavicular fossa.
N3 – Metastasis in lymph node (s)* > 6 cm and/or
to supraclavicular fossa*
Supraclavicular zone or fossa is relevant to the
staging of nasopharyngeal carcinoma and is the
triangular region which is defined by three points.
1. The superior margin of the sternal end of the
clavicle
2. The superior margin of the lateral end of the
clavicle
3. The point where the neck meets the shoulder.
Q 40. What is the importance of the “U” and “L”?
When the lower lymph nodes namely level 4 and
5, below the lower border of the cricoid cartilage
are involved the prognosis is bad.
Q 41. What percentage of occult metastasis, the
primary identification is possible?
Roughly in 1/3rd cases primary can be identified.
Q 42. Why primary is nonidentifiable in some
cases? (PG)
Possibly because of the spontaneous involution of
the unknown primary.
Q 43. If primary is not identified in the given case
would you recommend surgery if the report is
coming as SCC?
• Yes. A neck dissection is recommended if the
nodes are resectable
• A neck dissection removes additional ipsilateral
cervical nodes.
Q 44. What are the conditions where neck
dissections are valuable? (PG)
Conditions in which neck dissections are recommended
1. Squamous cell carcinoma
2. Salivary gland tumors
3. Thyroid carcinoma
4. Melanoma.
Q 45. What type of neck dissection is recommended?
Modified neck dissection may be appropriate.
Q 46. What are the indications for radiotherapy
after a modified neck dissection?
Indications for radiotherapy after a modified neck
dissection:
• If more than two lymph nodes contain metastasis
• Nodes at two or more levels contain metastasis
• Extracapsular spread of metastasis.
Q 47. What are the types of neck dissection?
The neck dissections may be classified as –
• Radical neck dissection (RND)—classical Crile
procedure (level I–V nodes removed)
• Modified radical neck dissection (MRND)
(described by Bocca) preserves one or more
of the following structures—spinal accessory
nerve, internal jugular vein and sternomastoid
muscle—type I, type II, type III
Type I—spinal accessory alone preserved
Type II—spinal accessory and sternomastoid
preserved
Type III—spinal accessory, sternomastoid and
internal jugular vein are preserved.
• Functional neck dissection (level II–V)—
preserving sternomastoid, internal jugular vein
and spinal accessory nerve
• Selective neck dissection—here one or more
lymph node groups are preserved –
1. Supraomohyoid neck dissection (removal of
level I–III)
2. Posterolateral neck dissection (removal of
level II, III, IV, V)
3. Lateral neck dissection (removal of level II, III,
IV)
4. Anterior compartment dissection (removal
of level VI).
Cervical Metastatic Lymph Node and Neck Dissections
305
Q 48. What is the difference between modified
radical neck dissection and functional neck
dissection?
• Modifiedneckdissectionalwayspreservesspinal
accessory nerve
• Functional neck dissection always preserves
sternomastoid muscle, the internal jugular vein
and spinal accessory nerve.
Q. 49 What are the structures removed in radical
neck dissection?
En-bloc removal of fat, fascia, and lymph nodes from
level I to level V.
They include the following:
• Two muscles—sternomastoid and omohyoid
• Two veins—internal jugular vein and external
jugular vein
• Two nerves—spinal accessory nerve and
cervical plexus
• Twoglands—submandibularsalivaryglands and
tail of parotid
• Prevertebral fascia.
Q 50. What is extended radical neck dissection? (PG)
This refers to the removal of one or more additional
lymph node groups and/or nonlymphatic structures
not encompassed by the radical neck dissection.
This may include the parapharyngeal and superior
mediastinal lymph nodes. The nonlymphatic
structures may include the carotid artery, the
hypoglossal nerve, the vagus nerve and the paraspinal
muscles. This is not an operation for occult primary.
Q. 51. What is the prognosis if the primary tumor
is never found? (PG)
This won’t influence the prognosis. If the primary
tumor is small or occult, it will be probably included
in the field of the postoperative irradiation and
cured by such treatment.
Prognosis is determined by whether or not the
tumor recurs or whether it metastasizes (metastasis
to lungs, bone or liver).
Q 52. How will you summarize the treatment
for SCC occult metastasis? [treatment of adenocarcinoma, poorly differentiated carcinoma and
poorly differentiated neoplasms are already given
above]
Summary of treatment for squamous cell
carcinoma metastasis from occult primary
It is treated according to the N stage:
N 1 – MRND (surgery is the treatment of
all N1 nodes) RT (radiotherapy) if
positive margins, capsular invasion and
multiple level nodes irradiate neck and
all potential sites of primary
N 2a and – Mobile → RND followed by RT, Fixed
N2b → RT followed by RND
N 2c – Bilateral RND followed by bilateral RT
N 3 – Resectable → RND followed by RT +
Chemo (controversy)
Unresectable → RT followed by RND
when it becomes resectable.
RND: Radical neck dissection
RT: Radiotherapy
Note: Regarding radiotherapy:
1. Radiotherapy is given for contralateral neck
nodes if primary is nasopharyngeal carcinoma.
2. Level II lymph nodes alone—primary is likely to be
nasopharynx and RT is preferred forsuch cases.
Q 53. What are the incisions used for neck
dissection? (Fig. 24.2) (PG)
1. Macfee incision: It consists of 2 horizontal
limbs. The first begins over the mastoid curving
down to the hyoid bone, and up again to the
chin, the second horizontal incision lies about
306
Clinical Surgery Pearls
2 cm above the clavicle from the anterior border
of the trapezius to the midline.
2. Schechter incision: It has a vertical limb and
horizontal limb. The vertical comes from the
mastoid process to the point where trapezius
meets the clavicle along the anterior border
of the trapezius. The horizontal, starting from
the middle of the vertical to the prominence of
thyroid cartilage.
3. The classical incision by Crile: It is a Y-shaped
incision with the upperlimbs ofthe“Y”reaching
posteriorly to the mastoid and anteriorly to the
Fig. 24.2: Neck incision series (A) Modified Crile incision for neck dissection (B) Martin neck incision (‘double Y’)
(C) MacFee neck incision (D) Schechter neck incision
A B
C D
Cervical Metastatic Lymph Node and Neck Dissections
307
chin. The stem of the “Y” reaches down to the
middle of the posterior triangle.
4. Martin incision: “Double Y”incision.
Q 54. What is the most poorly vascularized area
of skin in the neck and why? (PG)
• The middle of the neck laterally over the
common carotid artery
• The blood supply to the skin comes down
from the face, up from the chest, around from
trapezius and from the external carotid on the
other side
• Avoid a vertical incision over this area so that
a carotid artery rupture can be avoided
• Avoid three point junctions in the center of the
neck.
Q 55. What are the complications of neck
dissection? (PG)
Complications of neck dissection
1. Bleeding
2. Pneumothorax
3. Raised intracranial pressure (avoid pressure
dressings, use mannitol if required)
4. Wound breakdown
5. Infection
6. Necrosis of the skin flap
7. Seroma (use suction drain)
8. Rupture of the carotid artery
9. Chylous fistula (thoracic duct injury)
10. Frozen shoulder (due to accessory nerve damage)
—difficulty to abduct the arm.
Q 56. What precautions are taken to prevent
rupture of carotid artery? (PG)
• The carotid sheath should be protected either
by a muscle flap or a free dermal graft
• The commonly used muscle flap is levator
scapulae
• Use horizontal incisions
• Avoid three point junctions in incisions.
Q 57. What is the sequencing of bilateral neck
dissection and its prognosis? (PG)
• The presence of bilateral neck nodes at presentation is a bad prognostic sign
• Five year survival rate falls to about 5%
• Theusualpracticeofstaged neck dissection is now
changing to simultaneous bilateral neck dissection
• Themostfearedcomplicationafterbilateralneck
dissection is increased intracranial pressure
• Tying one internal jugular vein produces threefold increase in the intracranial pressure
• Tying the second side produces five-fold
increase in intracranial pressure
• However,the pressure tendsto fall over a period
of 8 days (the pressure fall is rapid within the 1st
12 hours).
Q 58. How will you avoid this complication of
increased intracranial tension? (PG)
1. Lumbar drain (removal of CSF)
2. Nursing the patient in the sitting position
3. Infusion of mannitol
4. Avoiding pressure dressings.
25 Carcinoma Tongue with
Submandibular Lymph Node
Case
Case Capsule
A 65-year-old male patient who is addicted to pan
chewing and smoking presents with nonhealing
ulcer in the right lateral aspect of the tongue. He
has profuse salivation and carries a handkerchief
for wiping the saliva. There is a pad of cotton wool
in the right ear, which he claims to take care of his
earache. He has difficulty in protruding the tongue
out. He has slurring of speech. There is offensive
smell when he opens his mouth. The submandibular
lymph node on right side is enlarged firm and mobile
of about 2 × 1 cm size. The jugulodigastric nodes on
both sides are enlarged, firm and mobile.
Checklist for history
1. History of chewing tobacco
2. History of smoking tobacco
3. History of alcoholism
4. History of tooth extraction followed by failure of
the socket to heal
5. History of unexplained tooth mobility
6. History of difficulty in wearing dentures
7. History of difficulty in opening the mouth and
protrusion of the tongue
8. History of difficulty in swallowing
9. History of excessive salivation
10. History of earache.
Checklist for clinical examination
1. Ask for ear pain or otalgia [Irritation of the lingual
nerve is referred to the auriculotemporal nerve]—
Cotton wool pad in the ear of the patient
2. Slurring of speech, when tongue is involved
3. Look for inability to protrude the tongue [ankyloglossia]
4. Ulcer that bleeds on touch
5. Look for profuse salivation which is due to the
irritation of nerve fibers of taste and as a result of
difficulty in swallowing
6. Look for deviation of the tongue indicating
involvement of the nerve supply to half of the
tongue [hypoglossal nerve]
7. Look for induration of the tongue when the
tongue is inside the mouth
8. Palpate the back of the tongue while the patient
sits on a stool
9. Tumors of posterior 3rd of tongue will spread to
tonsil and pillars of the fauces
10. Examine the cheek, gums, floor of the mouth,
trigone [retromolar] area and tonsils for a second
primary
11. Infiltration of the mandible causes pain and
swelling of the jaw
Contd...
Carcinoma Tongue with Submandibular Lymph Node
309
f. Significant metastatic lymph node in the
submandibular region.
Q 2. What are the differential diagnoses?
Differential diagnoses of carcinoma tongue
a. Dental ulcer [caused by irritation of tooth/denture]
b. Tuberculous ulcer—multiple small-grayish yellow
ulcers with undermining edges
c. Aphthous ulcer—small painful ulcer seen on the
under surface of the side of the tongue
d. Gumma—[very rare nowadays]
e. Chancre
f. Nonspecific glossitis.
Q 3. What is the most common malignancy of
the tongue?
Squamous cell carcinoma.
Q 4. What are the other malignancies possible in
the tongue other than squamous cell carcinoma?
a. Malignant melanoma
b. Adenocarcinoma.
12. Look for lymph nodes of the tongue namely, tip
to the submental and jugulo-omohyoid, margin to
the submandibular and upper deep cervical and
from the back to the jugulodigastric and juguloomohyoid
13. Remember the decussation of lymphatics of the
tongue and therefore the nodes of the other side
of the neck may be involved
14. Carcinoma tongue is a systemic disease, and
therefore look for metastasis especially pulmonary
15. Look for precancerous conditions and lesions.
Q 1. Why this is carcinoma tongue?
a. Elderly patient with an ulcer in the tongue
having raised and everted margins
b. There is induration on palpation which is in favor
of malignancy
c. Profuse salivation
d. Ankyloglossia
e. Offensive smell of malignant ulcer
Contd...
310
Clinical Surgery Pearls
Q 6. What are the macroscopic types of oral
cancers?
Macroscopic types of oral cancers
• Exophytic—less aggressive
• Ulcerative
• Combination.
Q 7. What are the pathological types of squamous
cell carcinoma?
Types of squamous cell carcinoma
• Verrucous—No lymph nodes
• Basaloid SCC—Advanced disease (Metastasis may
be there)
• Sarcomatoid—Lethal (Rapidly growing polypoidal
cancers).
Q 8. What are the peculiarities of verrucous
carcinoma?
• It is a controversial subject
• Presents as exophytic, whitish warty or
cauliflower-like growth
• Radiotherapy in verrucous carcinoma results in
a recurrence with anaplastic pattern than the
original primary
• Radiotherapy induces anaplastic transformation
• It seems that verrucous carcinoma already
contain foci of more malignant cells before
radiotherapy
• There is minimal invasion and induration.
• The lesions is densely keratinized and presents
as soft white velvety area
• Lymph node metastasis is late
• It is a low grade squamous cell carcinoma
• Most verrucous carcinomas are suitable for
excision and that is the treatment of choice.
Q 5. What are the investigations for the
management?
Investigations for oral carcinoma
1. Incisional biopsy of the ulcer under local
anesthesia for confirmation of the diagnosis—
biopsy should include the most suspicious area
along with normal adjacent mucosa. Areas of
necrosis and gross infection should be avoided
2. FNAC of the lymph node
3. Radiography—Orthopantomogram (OPG)—
provides information regarding the entire
mandible, but limited in its ability to evaluate the
symphysis and lingual cortex
4. OPG may be supplemented with dental occlusal
and intraoral X-rays
5. CT
• Indicated in patients with trismus
• Lesions abutting the mandible
• Where marginal mandibulectomy is planned
• To evaluate the clinically negative neck
• Patients with large nodes to look for carotid
artery involvement
• Itis very useful forthe assessment of pterygoid
regions
6. MRI scan for assessing the soft tissue spread
and perineural involvement. It is very useful for
tongue for assessing the extent of cancer. It is also
useful for other oral and oropharyngeal cancers.
Its great advantage over CT is that the image is
not degraded by the presence of metallic dental
restoration
7. Ultrasound of the neck and abdomen—
ultrasound guided aspiration of the neck is useful
in surveillance of patients with clinically NO neck
after treatment
8. X-ray chest for all patients
9. Dental consultation if radiation is planned
10. Assessment of the performance status (See chart
section)
11. Hb, full blood count, nutritional status, LFT and RFT.
Carcinoma Tongue with Submandibular Lymph Node
311
Q 9. What are the modes of spread of oral cancer?
1. Local spread to adjacent structures—soft tissues,
muscles, bone and neurovascular structures
2. Lymphatic spread—the first echelon lymph
nodes of primary SCC of the oral cavity are in the
supraomohyoid triangleoftheneck (LevelI, II, III)
3. Distant metastasis—exceedingly rare (lungs and
bones).
Note: Skip metastasis from primary carcinoma
may occur in 15% of patients of carcinoma tongue
without involvement of first echelon lymph nodes.
Q 10. Which oral cancer is having highest
incidence of nodal metastasis?
Carcinoma of the tongue, followed in descending
order by:
• Tumors of the floor of the mouth
• Lower alveolus
• Buccal mucosa
• Upper alveolus
• Hard palate.
Q 11. What is the mechanism of involvement of
mandible?
• It is involved by infiltration through its dental
sockets
• Throughdentalporesontheedentulous alveolar
ridge.
Q 12. What are the etiological factors for oral
cancer?
Etiological factors for oral cancer
A. Lifestyle habits
a. Tobacco (smoked or smokeless)
(Synergistic effectofsmokingandchewingoftobacco)
b. Betel nut
c. Alcohol
d. Human papilloma virus (HPV)
– Detected in 60–90% cases of oral cancer
– Present in 40% of normal oral cavity (direct
link between HPV and oral cancer remains to be
established)
e. Epstein-Barr virus
B. Dietary factors
a. Vitamin A (protective role)
b. Fresh fruits and vegetables
c. Iron deficiency anemia (Plummer-Vinson
syndrome) (SCC of hypopharynx and oral cavity)
C. Other risk factors
a. Poor dental hygiene
b. Ill-fitting dentures (chronic irritation).
The six S—Spices, Sprit, Sepsis, Sharp tooth,
Syphilis and Smoking.
Q 13. What is the risk of tobacco chewing for oral
cancer?
• Tobacco chewing, the risk is 8 times for buccal
cancer
• With quid it increases to 10 times
• If the quid is kept overnight, the risk increases
to 30 times
• Alcohol has synergetic effect with tobacco.
Q 14. What are the ingredients of tobacco
chewing?
It contains the following:
• Betel leaf
• Areca nut
• Smoked lime
• Catechu
• Condiments.
Contd... Note: It is commercially available as Pan masala.
Contd...
312
Clinical Surgery Pearls
Q 15. What is quid (Night quid)?
The above ingredients are kept in the gingivolabial
sulcus during night gives kick throughout night.
This is called a night quid.
Q 16. Which component of the chewing is
responsible for the premalignant lesions?
The chewing habits vary from place-to-place. The
usual ingredients are: betel leaf, lime, betel nut
and tobacco. The most important carcinogen
is tobacco. The betel nut has got two alkaloids
namely, arecoline and tannins. The arecoline
stimulate collagen synthesis and proliferation of
fibroblasts. The tannins stabilizes collagen fibrils.
Q 17. What is the action of alcohol?
The following actions are there for the carcinogenesis:
• Promoter
• Irritant
• Solvent—increases the solubility of carcinogen
• Alcohol suppresses the efficiency of the DNA
repair after exposure to nitrosamine compounds.
Q 18. What are the premalignant lesions of the
oral cavity?
Lesions of the oral cavity associated with an increased
risk of malignancy:
Precancerous lesions
a. Leukoplakia
b. Erythroplakia
c. Chronic hyperplastic candidiasis
Precancerous conditions
a. Oral submucous fibrosis
b. Syphilitic glossitis
c. Sideropenic dysphagia
Doubtful association
a. Oral lichen planus
b. Discoid lupus erythematosus
c. Dyskeratosis congenita.
Note:
• Precancerous lesions—These are morphologically altered tissue in which cancer is more
likely to occur than in its apparently normal
counterpart.
• Precancerous conditions—These are generalized states associated with significantly
increased risk of cancer.
Q 19. What is the WHO definition of leukoplakia?
Any white patch or plaque that cannot be
characterized clinically or pathologically as any
other disease.
Clinically present as white or gray/soft or crusty
lesion.
Q 20. What is the natural course of leukoplakia?
It may:
• Persist
• Regress
• Progress
• Recur.
Q 21. Which type of leukoplakia is dangerous?
There are two types of leukoplakia:
• Nodular
• Homogenous.
Speckled or nodular leukoplakia, which are the most
likely ones that will turn malignant.
Q 22. What are the pathological changes in
leukoplakia?
Pathological changes in leukoplakia
• Hyperkeratosis
• Parakeratosis
• Acanthosis.
Carcinoma Tongue with Submandibular Lymph Node
313
Q 23. What is the incidence of malignant change
in leukoplakia?
Incidence of malignancy in leukoplakia
• Overall 5% risk of malignant transformation
• More than 10 years duration – 2.4%
• More than 20 years duration – 4%
• Less than 50 years of patient's age – 1%
• Between 70 and 89 years – 7.5%
Note: Leukoplakia of the floor of the mouth and
ventral surface of the tongue has high incidence
of malignant change due to the pooling of
carcinogens in the floor of the mouth.
Q 24. What are the early clinical features of
malignancy in leukoplakia?
Clinical features of malignancy in leukoplakia
• Nodularity and thickness
• Ulceration
• Rolled margins
• Growths
• Indurated areas.
Q 25. What is the management of leukoplakia?
• Most of cases of leukoplakia will disappear if
alcohol and tobacco consumption ceases—ask
the patient to stop tobacco
• 1 year after the patient stops smoking and
drinking alcohol, leukoplakia will disappear in
60% of cases
• All lesions are biopsied (Biopsy from suspicious
area—ulceration, induration and hyperemia)
• If required surgical excision/CO2
laser may be
used and the small defects are closed and the
larger defects are left to epithelialize
• Regular follow-up at 4 monthly intervals.
Q 26. What is hairy leukoplakia?
White friable lesions of the tongue seen in AIDS are
called hairy leukoplakia.
Q 27. What is the WHO definition of erythroplakia?
Any lesion of the oral mucosa that presents as bright
red velvety plaques, which cannot be characterized
clinically or pathologically as any other recognizable
condition.
Q 28. What is the management of erythroplakia?
All lesions are excised because of the high incidence
of malignancy.
Q 29. What is chronic hyperplastic candidiasis?
Dense chalky plaques of keratin which are more
opaque than noncandidal leukoplakia. These
lesions are seen commonly in commissures.
Here, there is invasive candidal infection with an
immunological defect, there is high incidence of
malignant change.
Q 30. What is oral submucous fibrosis?
In this condition, fibrous bands form beneath the
oral mucosa and these bands progressively contract
ultimately resulting in restriction of opening of
the mouth and tongue movements. This entity
is confined to Asians. The etiology is obscure.
Hypersensitivity to chilli, betel nut, tobacco and
vitamin deficiencies are implicated. Slowly growing
squamous cell carcinoma is seen in 1/3rd of patients.
Q 31. What are the features of oral submucous
fibrosis (SMF)?
Features of oral submucous fibrosis
• SMF is a high-risk precancerous condition
• There is strong association between SMF and
chewing areca nut
• It can affect any part of the oral mucosa
Contd...
314
Clinical Surgery Pearls
• Palpable fibrous bands over the buccal mucosa,
retromolar area and rima oris
• Restriction of mouth opening—Trismus (in severe
case impossible to open the mouth)
• It will not regress with cessation of areca nut
chewing
• It may spread to involve wider areas.
Q 32. What is the histology of oral submucous
fibrosis?
• Juxta epithelial fibrosis with atrophy or hyperplasia of the overlying epithelium (fibroelastic
transformation initially)
• Areas of epithelial dysplasia are seen.
Q 33. What is the treatment of oral submucous
fibrosis?
• Intralesional injection of steroids
• Surgical excision and grafting (Note: this will not
prevent squamous cell carcinoma).
Q 34. What is syphilitic glossitis?
Syphilitic glossitis will produce the following changes:
Syphilitic glossitis
↓
Endarteritis
↓
Atrophy of overlying epithelium
↓
More vulnerable to irritants
↓
Squamous cell carcinoma (even in the absence of
leukoplakia)
Note:
• These changes are irreversible
• Thereisnospecific treatmentforsyphiliticglossitis
• The syphilis must be treated.
Q 35. What are the causes for glossitis?
Causes for glossitis
• Median rhomboid glossitis
• Geographic tongue
• Hairy tongue—It is only the appearance and not
the presence of hair
• Pernicious anemia—Hunter’s glossitis
• Agranulocytosis
• Pellagra (deficiency of B2).
Q 36. What are the causes for hairy tongue?
• Black hairy tongue occurs in response to some
antibiotics and antiseptics
• There is overgrowth of filiform papillae which
become stained black by bacteria, medication
or tobacco.
Q 37. What is median rhomboid glossitis?
It is characterized by the appearance of a rhomboid or
oval mass in the midline of the tongue, immediately
in front of the foramen cecum. The mass is slightly
raised, smooth and devoid of papillae. It is probably
as a result of candidal infection.
Q 38. What is geographic tongue?
• It is a condition of unknown etiology
• Red patches with yellow bordersform a pattern
on the dorsum of the tongue
• The pattern will change from day-to-day
• The condition startsin childhood and continues
throughout life
• Some cases remit spontaneously.
Q 39. What is sideropenic dysphagia (PlummerVinson syndrome/Paterson-Kelly syndrome)?
• Common in Swedish women
• Higher incidence of cancer of the upper
alimentary tract in this group
• Itisthe cause for higherincidence of oral cancer
in women in Sweden
Contd...
Carcinoma Tongue with Submandibular Lymph Node
315
• Of women with oral cancer 25% are sideropenic
• The pathogenesis may be similar to syphilitic
glossitis (as a result of epithelial atrophy)
• The iron deficiency anemia seen will respond
to treatment with iron supplements (the risk
of subsequent malignant change may not be
altered).
Q 40. In which type of oral lichen planus, there is
more risk for malignant transformation?
Atrophic and erosive lichen planus.
Q 41. What is dyskeratosis congenita?
This syndrome is characterized by:
a. Reticular atrophy
b. Nail dystrophy
c. Oral leukoplakia.
Q 42. What is the most common site of squamous
cell carcinoma in the tongue?
Middle third of the lateral margin of the tongue.
The incidences at various sites in the tongue are
given below:
• 25%—Anterior 1/3rd (lateral margin) × 2 (on
each side = 50%)
• 10%—Tip of the tongue
• 10%—Under surface of the tongue
• 5%—Dorsum of the tongue
• 25%—Posterior 3rd ofthe tongue (posterior 3rd
is not oral tongue).
Q 43. What are the clinical features of carcinoma
of the tongue?
Clinical features of carcinoma of the tongue
• Exophytic lesion with areas of ulceration
• Ulcer in the depth of fissure
• Superficial ulceration with infiltration
• Ankyloglossia (inability to protrude the tongue)
• Hypoglossal nerve palsy
• Regional node enlargement
• Earache
• Profuse salivation (inability to swallow and
increased salivation because of irritation of the
nerves of taste)
• Difficulty in speech
• Dysphagia
• Offensive smell (fetor).
Q 44. What is the lymphatic drainage of tongue?
Lymphatic drainage of the tongue
• Lymphatics from the tip of the tongue—to the
submental nodes and jugulo-omohyoid
• Lymphaticsfromthemargin—tothesubmandibular
nodes and upper deep cervical
• From the back of the tongue—to the jugulodigastric and jugulo-omohyoid
• There is decussation of lymphatic vessels.
Note: The lymph nodes of both sides of the neck
must be examined, even if the lesion is unilateral
since the lymphatic vessels are decussating.
Q 45. What is the AJCC staging of the oral cavity
tumors?
AJCC staging
Primary
Tis Carcinoma in situ
T1 Tumor< 2 cm
T2 Tumor> 2 cm to < 4 cm
T3 Tumor > 4 cm
T4a Moderately advanced local disease. Tumor
invades through cortical bone, inferior alveolar
nerve, floor of mouth, skin of face, that is chin or
nose. Tumor invades adjacent structures only.
Contd...
Contd... Contd...
316
Clinical Surgery Pearls
For example, Cortical bone (mandible or
maxilla) into deep extrinsic muscle of tongue
(genioglossus, hyoglossus, palatoglossus and
styloglossus), maxillary sinus, skin of face
T4b Very advanced local disease – Tumor invades
masticator space, pterygoid plates or skull base
and or encase internal carotid artery
Note: Superficial erosion alone of bone/tooth socket
by gingival primary is not sufficient to classify as T4.
Neck
N0 No clinically palpable node
N1 Single ipsilateral node < 3 cm
N2a Single ipsilateral node > 3 cm to 6 cm
N2b Multiple ipsilateral nodes < 6 cm
N2c Bilateral or contralateral nodes < 6 cm
N3 Nodes > 6 cm
Distant Metastasis
MX Distant metastasis cannot be assessed
M0 No distant metastasis
M1 Distant metastasis
Stage Grouping
Stage 0 Tis N0 M0
Stage I T1 N0 M0
Stage II T2 N0 M0
Stage III T3 N0 M0
T1 N1 M0
T2 N1 M0
T3 N1 M0
Stage IV A T4a N0 M0
(moderately T4a N0 M0
advanced T4a N1 M0
disease) T1 N2 M0
T2 N2 M0
T3 N2 M0
T4a N2 M0
Any T N2 M0
Stage IVB Any T N3 M0
Very advanced
T4b
Any N M0
Stage IVC Any T Any N M1
Metastatic
Disease
Q46. What is the surgical management of
carcinoma of the tongue?
• It consists of treatment of the primary lesion
and treatment of the metastatic nodes.
• Three dimensional excision is the treatment of
choice for the primary
a. Small lesions less than 2 cm size:
• Excise the lesion and the defect is left to
granulate and epithelialize
• Resection of less than one third of the
tongue does not require reconstruction
• It can also be treated by Brachytherapy by
iridium wires(this will preserve the tongue)
• CO2 laser also can be used for partial
glossectomy.
b. Lesions of more than 2 cm size:
• Hemiglossectomy is the minimum treatment
• Preserve one hypoglossal nerve (this will
give reasonable speech and the patient will
learn to swallow)
• For T1 and T2 lesions after glossectomy,
simple quilted splint skin graft is enough
c. Extensive lesion involving the floor of the mouth
and alveolus:
• Major 3dimensionalresectionby lip split and
mandibulotomy is required
Contd... Contd...
Contd...
Carcinoma Tongue with Submandibular Lymph Node
317
• Marginal mandibular resection may be
required
• Dissection of the neck on the same side is
also carried out
• This is followed by reconstruction with a
Radial forearm flap with microvascular
anastomosis(radial forearm flap is the work
horse of oral reconstruction). This flap is
useful if the volume defect is less than 2/3rd
of the original tongue
• A bulky flap may be required after total
glossectomy for a very large defect
Q 47. What is marginal mandibular resection?
Marginal mandibulectomy involves an incontinuity excision of tumor with a margin of
mandible and overlying gingiva. Mandibular
continuity is maintained and a much better cosmetic
and functional end result is achieved. A segment
of bone at least 1 cm thick must be left inferiorly.
Marginal mandibular resection is done if the tumor
reaches but does not invade the alveolus.
This is because of the peculiarity of the mode
of involvement of the mandible. It is involved by
infiltration through its dental sockets or dental
pores on the edentulous alveolar ridge. These
cells proceed along the root of the tooth into the
cancellous part of the mandible and then along
the mandibular canal.
Q 48. What are the contraindications for marginal
mandibulectomy?
• Radiological involvement of the bone
• Previous radiotherapy—cause osteoradionecrosis and fracture
• Retromolar primary lesion
• Deeply infiltrating gingivobuccal lesion with
paramandibular infiltration.
Q 49. What is commando operation?
It is an old operation where combined (composite)
excision of the primary tumor, block dissection
of the cervical lymph nodes and removal of the
intervening body of the mandible is done (it
was presumed previously that the spread to the
mandible is by lymphatics on its way to the regional
nodes. But now we know the method of spread
to the mandible and hence, the introduction of
marginal mandibulectomy).
Q 50. What is the management of neck nodes?
(Read the block dissection part in short case No:2)
• A modified radical neck dissection (MRND) is
recommended for N1 and N2 nodes.
• A supraomohyoid neck dissection (SOHND)
(clearance oflevel I, II, III nodeswith preservation
of sternocleidomastoid, internal jugular vein
and spinal accessory) and postoperative
radiotherapy has been advocated by some
authors for N1, Level I disease.
Q 51. Is there any role for elective lymph node
dissection (ELND) in N0 neck (no neck nodes)?
Yes.
• Occult nodal metastatic disease is present in
5–40% of oral cancers depending on T status
and grade of primary
• Clinical N0 neck should be treated by supraomohyoid neck dissection (SOHND), ifthe risk of
occult nodal metastasis is greater than 15–20%
in patients with T3/T4 primary
• Patient with T1/T2 tongue tumors and cancers
of the floor of the mouth more than 2 mm thick.
• It is also indicated if it is necessary to enter the
neck for resecting the primary
• In short neck individualsrequiring bulky flap for
oral reconstruction (to create space)
318
Clinical Surgery Pearls
Q 56. How is radiotherapy given?
• External beam radiotherapy
• Interstitial radiotherapy
• Combination of both.
Q 57. What is the dose of radiotherapy?
The total dose is 65–75 Gy to the primary and neck.
Q 58. What are the complications of radiotherapy?
Complications of radiotherapy
• Xerostomia
• Tissue edema
• Erythema
• Skin sloughing
• Ulceration
• Dental caries
• Osteoradionecrosis.
Q 59. What are the causes of death in carcinoma
tongue?
Causes of death in carcinoma tongue
• Inhalation and aspiration pneumonia
• Cachexia and starvation
• Hemorrhage from growth
• Hemorrhage from carotid artery when eroded by
metastatic lymph nodes
• Asphyxia secondary to pressure from lymph nodes
• Edema glottis.
• If the patients are unreliable for follow-up.
• In patients undergoing elective SOHND 24 to
31% will have histological evidence of lymph
node metastasis.
Q 52. If the neck nodes are pathologically positive
after SOHND, what next?
• If detected positive on the operating table,then
SOHND should be converted to RND/MRND
• If positive following surgery—subsequent RND
or postoperative radiotherapy.
Q 53. How to tackle the skip metastasis to level IV
which is seen in 15% patients with tongue cancer?
Extended SOHND is recommended by some group
to tackle this problem where the level IV nodes are
also removed.
Q 54. What is the management of bilateral nodal
metastasis?
Bilateral neck dissection with preservation of
internal jugular vein on one side.
Q 55. What are the indications for radiotherapy
for primary?
Indications for radiotherapy
• For early lesions of the tongue
• Early lesions of the buccal mucosa
• Patient is medically unfit
• Patient is unwilling for surgery.
26 Carcinoma of Gingivobuccal
Complex (Indian Oral Cancer)
Case
Case Capsule
A 60-year-old male patient addicted to chewing
tobacco for the last 35 years and drinking alcohol
presents with history of tooth extraction with
subsequent failure of the socket to heal in the
right lower molar region for the last 6 months.
On examination there is an indurated ulceroproliferative lesion extending from the tooth
extraction socket in the first molar region of the
lower gingiva to the gingivobuccal sulcus of
5 × 3 cm size. This lesion involves the overlying skin
of the cheek resulting in 3 sinuses. The patient
has difficulty in opening the mouth (trismus).
The anterior pillar of the fauces and retromolar
trigone seems free. The submandibular lymph
node is enlarged of about 2 × 1 cm size and hard in
consistency. There are 3 leukoplakic patches seen
on the buccal mucosa on left side.
Read the checklist for the history and examination of
carcinoma tongue.
320
Clinical Surgery Pearls
Q 6. What are the areas involved by buccal cancers?
• The gingivobuccal sulcus
• Retromolartrigone
• Lower and upper alveolus
• Buccal mucosa.
Q 7. What is Indian oral cancer?
The buccal mucosa and gingiva are more often
affected by cancer as a result of placement of the
tobacco quid in the oral cavity. This cancer of the
gingivobuccal complex is described as the Indian
oral cancer.
Q 8. What is the commonest age group affected?
It is 5th to 7th decade.
Q 9. What is the extent of buccal mucosa?
The buccal mucosa extends from the upper
alveolar ridge down to the lower alveolar ridge,
from the commissure anteriorly to the mandibular
ramus and retromolar region posteriorly.
Q 10. What is the cause for trismus in this case?
Infiltration of the muscles by carcinoma is responsible
for trismus in this case. The following muscles may be
involved in carcinoma of the buccal mucosa:
• Buccinator
• Pterygoid
• Masseter
• Temporalis.
Q 11. What are the causes for trismus?
Causes for trismus
• Submucous fibrosis
• Invasion of musclesby carcinoma (mentionedearlier)
• Tetanus—Risus sardonicus (painful smiling)
• Parotitis
• Dental abscess
• Erupting wisdom tooth
• Peritonsillar abscess.
Q 1. What is the most probable diagnosis in this
case?
Carcinoma of the gingivobuccal complex.
Q 2. What are the clinical points in favor of
carcinoma?
• History oftooth extraction followed by failure of
the socket to heal
• The indurated ulceroproliferative lesion with
everted margins
• Involvement of the overlying skin with sinuses
• Presence of hard submandibular lymph node
• Trismus
• Presence of leukoplakia
• History of pan chewing and smoking.
Q 3. What is the definition of oral cavity?
The term oral cavity refers to the following:
Oral cavity
• Lips
• Buccal mucosa
• Alveolar ridges (upper and lower gingiva)
• Retromolar trigone
• Hard palate
• Floor of the mouth
• Anterior two-thirds of the tongue (oral or mobile
tongue).
Note: Cancer of the lip behaves clinically like skin
cancer, and therefore not discussed with oral cavity
lesion.
Q 4. What is the incidence of oral cancers in India?
About 16 to 28 per 100,000 population [ICMR].
Q 5. What is the commonest oral cancer in India?
In India, carcinoma of the buccal mucosa is the
commonest oral cancer constituting about 50 to
83% of oral cancers. In the West, tongue and floor
of the mouth are the commonest sites [30%].
Carcinoma of Gingivobuccal Complex (Indian Oral Cancer)
321
It can summarized as:
In this case the staging is:
T4, N1, M0 → stage IV A.
Q 17. What are the investigations required?
Read the investigation chartfor carcinoma tongue.
(Confirm the diagnosis by biopsy from the most
suspicious area avoiding the areas of infection and
necrosis).
Q 18. What is the management of stage IV disease?
• Generally stage I and II (Early) diseases are
managed by Surgery/Radiotherapy (Either
surgery or radiotherapy)—No radiotherapy in
gingivobuccal complex due to close proximity
of the tumor to bone and risk of radio necrosis.
• StageIII and IV (Advanced) are managed by Radical
Surgery and Reconstruction and Radiotherapy
(Surgery and Radiotherapy are combined).
• Surgery isthe treatment of choice for all alveolar
carcinomas except for patients unfit for surgery.
Q 19. What is the surgical treatment if the carcinoma is confined to the buccal mucosa?
• It is excised widely including the underlying
Buccinator muscle.
• This is followed by split—skin graft.
Q 20. What is the surgical management of more
extensive lesion?
• Three dimensional excision and reconstruction.
Q 21. What are the flaps available for reconstruction
after 3dimensional excision of oral cancer? (PG)
Flaps available for reconstruction
• Free radial forearm flap—isthe work - horse of oral
reconstruction
• Buccal fat pad—For small intraoral defects of upto
3 × 5 cm – Used for reconstruction of maxillary
Q 12. What is the grading of trismus? (PG)
Depending on the degree of mouth opening
possible it is graded into 4 groups:
Grading of trismus
• Grade I – more than 35 mm
• Grade II – 26 to 35 mm
• Grade III – 16 to 25 mm
• Grade IV – < 15 mm
Q 13. What is the cause for sinus in this case?
Orocutaneous fistula secondary to malignant
infiltration.
Q 14. What is retromolar trigone?
• The retromolartrigone is defined asthe anterior
surface of the ascending ramus of the mandible
• It is triangular in shape
• The base issuperior behind the 3rd upper molar
tooth
• The apex is inferior behind the 3rd lower molar
tooth.
Q 15. What are the special problems of carcinoma
of the retromolar trigone? (PG)
• Tumors at this site may invade the ascending
ramus of the mandible
• I t may spread upwards to involve the
pterygomandibular space
• A lip split and mandibulotomy are needed to
gain access to this region
• Mandibulectomy isrequired for clearance ofthe
pterygoid region
• The resultantdefectismanagedbymasseter and
or temporalis muscle flap.
Q 16. What is the staging in this case?
Read the staging of oral cancers given in Carcinoma
Tongue Chapter. Contd...
322
Clinical Surgery Pearls
defects, hard and soft palate defects, cheek and
retromolar defects
• Temporalismuscle flap(forlargerdefects alongwith
buccal fat pad)
• Forehead flap—now rarely used because of the
poor cosmetic outcome.
Q 22. What is the management if the mandible is
radiologically not involved?
Marginal mandibulectomy (Read carcinoma
tongue).
Q 23. What are the contraindications for marginal
mandibulectomy? (PG)
Contraindications for marginal mandibulectomy
• Gross clinical involvement of mandible
• Radiological involvement of the mandible
• Deeply infiltrating lesions of the gingivobuccal
sulcus with paramandibular infiltration
• Previous radiotherapy (osteoradio necrosis)
• Retromolar lesions (clearance of the pterygoid
region is not possible).
Q 24. In the present case there is gross clinical
involvement of the mandible and paramandibular
infiltration. What is the surgical management?
Hemimandibulectomy or segmental mandibulectomy is required, along with the 3 dimensional
excision and a modified radical neck dissection (MRND).
Q 25. What is the deformity produced by the
resection of the anterior arch of mandible? (PG)
Andy Gump deformity.
Q 26. What are the bony substitutes available for
reconstruction after mandibulectomy? (PG)
Reconstruction with osteomyocutaneous flap or
free microvascular bone graft immediately.
• Radial forearm flap with a section of radius
• Compound groin flap based on the deep
circumflex iliac vessels
• Free fibula flap
• Corticocancellous grafts harvested from iliac
crest (packed into mesh trays—Titanium trays)
• Rib grafts.
Q 27. What is the soft tissue cover for the reconstructed bone? (PG)
• For the microvascular free flaps the associated
skin is used (compound groin flap based on the
deep circumflex iliac vessels)
Pectoralis major muscle flap (this is wrapped
around the bone graft and sutured on the labial
aspect).
Q 28. What are the indications for surgery in
general for oral cancer?
Indications for surgery in oral carcinoma
1. Tumors on alveolar process
2. Very large mass when there is invasion of bone
3. Nodal involvement—primary andnodes are treated
surgically
4. Multiple primary tumors—surgery is preferred
5. Verrucous carcinoma.
Q 29. Is there any role for radiotherapy as a
primary modality in Early gingivobuccal complex?
No. (Because of the proximity to mandible)
Q 30. What is the role for preoperative radiotherapy
in Advanced gingivobuccal complex?
Indications for preoperative radiotherapy in
advanced gingivobuccal complex
• Inoperable diseases
• Patient is unfit for surgery
• Patient is unwilling for surgery
• Down staging is possible.
Contd...
Carcinoma of Gingivobuccal Complex (Indian Oral Cancer)
323
Q 31. What are the indications for postoperative
radiation therapy to the primary?
Indications for postoperative radiation
therapy to the primary
• T3/T4 primary
• Residual microscopic tumor
• Positive surgical margins
• Gross residual tumor after resection.
Q 32. What are the indications for adjuvant
radiation to the neck after radical neck dissection?
Indications for adjuvant radiation to the neck after
radical neck dissection
• More than 2 positive nodes
• 2 or more levels of nodes involved
• Extracapsular spread.
Q 33. What are the indications for elective neck
irradiation?
ElectiveneckirradiationisusedforN0orN1neckifthe
treatment of primary with radiation therapy is effective.
Q 34. What is the survival for stage III and IV
disease after treatment? (PG)
• With radiation or surgery alone the survival for
stage III is 41% and Stage IV is 15%.
• When surgery is combined with postoperative
radiation therapy these rates increase to 60%
and 35%.
Q 35. What is the management of inoperable cases?
Inoperable cases are managed by radiation therapy
with or without chemotherapy.
Q 36. What are the indications for chemotherapy? (PG)
Indications for chemotherapy
• Palliation for advanced oral cancer
• Recurrent oral cancers
• Verrucous carcinoma
• Adjuvant
• Neoadjuvant
• Concurrently with radiation: chemoradiation.
Q 37. What is the advantage of chemoradiation?
• Improve the locoregional control
• Prevent metastasis.
Q 38. What is the disadvantage of chemoradiation?
• Significant treatment related morbidity.
Q 39. What is the contraindication for chemotherapy?
Poor performance status(Read the Chart andTable
section).
Q 40. What are the chemotherapeutic agents used?
Cisplatin—based combination chemotherapy is
more effective than single agent chemotherapy
(Cisplatin and 5-FU).
The commonly used agents either alone or in
combination are:
• Methotrexate
• 5-fluorouracil (5-FU)
• Cisplatin
• Bleomycin
• Ifosfamide.
Q 41. What are the poor prognostic factors?
Poor prognostic factors
• Stage at presentation (single most important)
• Lymph node metastasis
• Number of lymph nodes involved
• Extracapsular spread in the node
• Tongue cancer has poor prognosis compared to
other subsites.
Q 42. What is the survival figure for early and
advanced stages? (PG)
• Stage I andII(early)—5 yearsurvival of 31–100%.
• StageIII andIV(advancedstages)—5yearsurvival
Contd... of 7–41% depending on the site of the disease.
Contd...
27 Parotid Swelling
Case
Case Capsule
A 45-year-old male patient presents with painless
enlargement of the right parotid gland. On
examination, there is a swelling of about 4 × 3
cm size irregular in shape and occupying the
hollow between the mandible and mastoid. It is
firm in consistency, deep to the parotid fascia, and
superficial to the masseter muscle. The swelling
raises the right ear lobule. The facial nerve is
intact. The superficial temporal artery is palpable
above the swelling. There are no palpable ipsilateral
nodes. The patient is apparently healthy.
Read the diagnostic algorithm for a neck swelling
Checklist for history
1. History of systemic diseases responsible for
sialadenosis like—DM, drugs (antiasthmatic,
Guanethidine) endocrine disorders, alcoholism,
pregnancy, bulimia (eating disorders)
2. History of exposure to mumps
3. History of collagen diseases
4. History of salivary colic
5. History of increase in size during salivation
6. History of similar swelling on the contralateral side
7. History of recent illness and major surgery (acute
parotitis)
8. History of exposure to HIV (HIV associated
sialadenitis).
Checklist for examination
1. Look for obliteration of the hollow below the ear
lobule
2. Look for fixity to masseter
3. Bimanual palpation of the deep lobe with one
finger inside at the tonsillar region and other hand
externally
4. Bidigital palpation of the Stensen’s duct (thumb
externally and index finger internally)
5. Look for lymph nodes—Preauricular, parotid and
submandibular nodes
6. Look for movements of the jaw
7. Look for facial nerve palsy
8. Examine the oral cavity—Orifice of Stensen’s duct,
Tonsil (whether pushed medially or not)
9. Always examine other ipsilateral salivary glands and
other contralateral salivary glands. Contd...
Contd...
Parotid Swelling
325
Q 3. What is the classical site of parotid swelling?
• Below, behind and slightly in front of the ear lobule
• It obliteratesthenormal hollowbelowthe lobule
of the ear.
Q 4. What is the clinical test by which you say that
the swelling is deep to parotid fascia?
The parotid fascia is stretched by asking the patient to
open the mouth. If the swelling is deep to the parotid
fascia, the swelling will become less prominent.
Q 1. What is the most probable diagnosis?
Parotid swelling.
Q 2. What are the points in favors of parotid
swelling?
The following points characterize the swelling:
1. Deep to the parotid fascia
2. It is superficial to masseter
3. It is raising the ear lobule
4. Occupying the normal anatomic area of the parotid.
326
Clinical Surgery Pearls
Q 10. What is the commonest cause of acute
parotitis?
Mumps (caused by paramyxovirus) manifested by
• Bilateral or unilateral parotid swelling (double chin
appearance due to the spreading down of the
edema)
• Fever
• Arthralgia
• Orchitis (rarely)
• Pancreatitis
• Thyroiditis
• Sensory neural hearing loss.
Q 11. Why should you examine the contralateral
side and other salivary glands?
Autoimmune diseases of the salivary gland like
Sjögren’s syndrome and Mikulicz’s syndrome will
cause symmetrical enlargement of the salivary glands.
Q 12. What is Mikulicz’s syndrome?
It is a combination of bilateral salivary and
lacrimal gland enlargement
• Symmetrical enlargement of salivary glands (one
gland alone is involved initially for a quite long time)
• Enlargement of lacrimal glands (bulge below
the outer end of the eyelids and narrowing of
the palpebral fissure)
• Dry mouth.
Q 13. What are the causes for Mikulicz’s syndrome?
• Sarcoidosis
• Leukemia
• Lymphoma
• Sjogren’s syndrome.
Q 14. What is Sjögren’s syndrome?
It is a rare autoimmune condition affecting the
salivary glands and it can occur in combination
with other autoimmune connective tissue disorders.
Q 5. Why do you say that the swelling is superficial
to masseter muscle?
Ask the patient to clench the teeth. This will contract
the masseter muscle. The parotid gland is superficial
to the masseter and therefore it will become more
prominent.
Q 6. What is salivary colic?
During salivation, there will be pain and increase
in size of the swelling, which is typically seen in
submandibular salivary duct stones.
Q 7. What is sialadenitis?
Inflammation of the salivary gland is called
sialadenitis it may be classified as:
• Acute bacterial sialadenitis—Seen in elderly
bedridden patients and neonates
• Chronic sialadenitis—Due to obstruction or
narrowing of the Stensen’s or Wharton’s duct by
a calculus or stricture.
Q 8. What are the manifestations of acute bacterial
sialadenitis?
It is associated with poor oral hygiene, dehydration,
general debilitation, etc. and clinically manifested
as:
• Sudden painful (tender) swelling of the parotid
gland
• Trismus
• Dysphagia
• Fever.
Q 9. What are the manifestations of chronic
sialadenitis?
• Recurrent parotid swelling especially during
eating
• Enlargement of the gland (rubbery hard)
• Stricture of the duct.
Parotid Swelling
327
The manifestations and associated conditions
seen in Sjögren’s syndrome.
Manifestation Condition
Deficient tear film Keratoconjunctivitis sicca
Deficient salivation and
gland enlargement
Xerostomia
Deficient tear and saliva Primary glandular sicca
syndrome
Deficient tear and saliva
along with hyperglobulinemic purpura, vasculitis, or Raynaud’s phenomenon or B cell lymphoma
Primary extra-glandular
sicca syndrome
Any of the above occur-ring
together with rheumatoid
arthritis, systemic lupus
erythematosus or other
recognizable connective
tissue disorders
Secondary Sjögren’s
syndrome
Q 15. What is the importance of oral cavity
examination in parotid swelling?
• To look for the orifice of the Stensen’s ducts
which is situated opposite the crown of the
second upper molar tooth—Look for blood
and pus.
• To palpate the mouth of the duct for any lumps
and induration.
• Gentle pressure on the gland externally may
bring out purulent discharge.
• The tonsil may be pushed medially when the
deep lobe of the parotid gland is enlarged.
• Bimanual palpation of the parotid gland—
One finger externally behind the ramus of the
mandible and one finger inside the mouth just
in front of the tonsil and behind 3rd molar tooth
internally.
Q 16. What is the anatomical position of the
parotid duct?
It is deep to the anterior border of the gland and
runs superficial to the masseter muscle. It then
curves inwards by piercing the buccinator to open
on the mucous membrane of the mouth opposite
the crown of the upper second molar tooth.
Q 17. How would you palpate the Stensen’s duct?
It is best done by a bidigital palpation by index
finger inside the mouth and thumb over the
cheek.
Q 18. What is the surface marking for Stensen’s
duct?
It lies about one fingerbreadth below the inferior
border of the zygomatic bone.
Q 19. What is the most important differential
diagnosis for a small parotid swelling?
Preauricular lymph nodes (enlargement secondary
to infection or metastasis).
Q 20. What are the primary foci for enlarged
preauricular lymph nodes?
Primary sites for preauricular node metastasis:
Drainage area for pre auricular node
• Forehead
• Scalp
• Eyelids
• Cheek
• External auditory meatus.
Q 21. What is the distinguishing clinical feature
of the lymph node?
It is the mobility of the lymph node—The preauricular lymph node is outside the capsule of the
gland and usually very mobile, unlike the tumor in
the parotid which has got restricted mobility.
328
Clinical Surgery Pearls
Q 22. What is parotid sandwich?
The facial nerve is passing through the substance
of the parotid gland, dividing the gland into a
superficial lobe and deep lobe. Therefore the gland
is called parotid sandwich.
Q 23. What is faciovenous plane of Patey?
The facial nerve is seen, superficial to the posterior
facial vein in the substance of the gland. This plane
is called faciovenous plane of Patey.
Q 24. What is Pes anserinus?
Pes anserinus means goose foot.
In the parotid gland the facial nerve divides into—
1. The temporofacial (runs sharply upwards)—two
divisions (temporal, zygomatic)
2. The cervicofacial—continues the course of the
parent trunk downwards, forwards and outwards—
three divisions (buccal, mandibular and cervical).
These divisions in turn divide to form the goose’s
foot (Pes anserinus).
Q 25. What is sociaparotidis?
It is nothing but accessory lobe of the parotid seen
just above the Stensen’s duct.
Q 26. Which type of facial palsy is seen in parotid
tumors?
Lower motor neuron type of facial palsy is seen
(involvement of both lower and upper half of the
face).
Q 27. What are the tests for facial palsy?
The tests for facial palsy are:
Test Manifestations
1. Ask the patient to show
his teeth
Angle of the mouth drawn
to the healthy side
2. Ask the patient to puff
out the cheeks
The paralyzed side
bellows out more than
the normal side
3. Ask the patient to shut
his eyes
Will not be able to close
the eyes on the affected
side and on attempting to
do so to the eyeball will be
seen to roll upwards
4. Ask the patient to move
his eyebrows upwards
T h e p a r a l y z e d s i d e
remains immobile
Note: The nasolabial fold and furrows of the eyebrow
are less marked on the affected side. The angle of
the mouth is drawn to the sound side.
Q 28. Will all the malignant tumors produce facial
palsy?
No.
Q 29. What are the clinical features of malignancy
in the parotid?
Clinical features of malignancy
a. Pain
b. Rapid increase in size
c. Very hard consistency
d. Facial palsy
e. Enlarged metastatic regional node
f. Skin involvement (skin tethering)
g. Fixity
h. Trismus—involvement of pterygoid muscle by deep
parotid lobe malignancy
Q 30. What is the commonest parotid swelling?
Pleomorphic adenoma (mixed parotid tumor).
Q 31. What percentage of tumors are benign in
parotid?
Site % of benign % of malignant
Parotid 80% 20%
Submandibular 50% 50%
Minor salivary gland 10% 90%
Sublingual 5% 95% Contd...
Contd...
Parotid Swelling
329
Q 32. What are the other benign tumors of the
parotid gland?
• Warthin’s tumors (papillary cyst adenoma
lymphomatosum)
• Oxyphylic adenoma (Oncocytoma).
Q 33. What are the features of Warthin’s tumor?
Clinical features of Warthin’s tumor
• 2nd most common benign tumor
• It is soft and sometimes fluctuant (cystic)
• Seen usually in males
• Seen after 40 years
• May be bilateral (10% bilateral)
• This tumor has no malignant potential.
Q 34. What is the origin of Warthin’s tumor?
The tumor probably arises from parotid tissue
included in the lymph nodes which are usually
present within the parotid sheath. Microscopically
it is lined by columnar epithelial cells supported by
lymphoid stroma.
Q 35. Is there any method of confirming Warthin’s
other than FNAC? (PG)
Yes. Tc 99m scintigraphy will reveal a hot spot. This is
due to the high mitochondrial content within the cell.
Q 36. What is oncocytoma? (PG)
They arise from oncocytes which are derived from
intralobular ducts or acini. They are usually seen in
minor salivary glands, nasopharynx and larynx in
the elderly males.
Q 37. What is the investigation of choice in parotid
tumors?
Fine needle aspiration cytology (FNAC).
Q 38. Why biopsy is contraindicated in parotid
tumors?
Biopsy is contraindicated because of the following
reasons:
1. Seedling of the tumor will occur
2. Chance for parotid fistula is there
3. Chance for facial nerve injury.
Q 39 What are the other investigations?
1. CT/MRIis taken to rule out deep lobe involvement
2. Chest X-ray to rule out metastasis.
Indications for CT
1. If deep lobe tumor is suspected
2. If extension to deep lobe is suspected
3. Trismus.
Indication for MRI
When facial nerve is involved.
Q 40. What is the WHO classification of parotid
neoplasms?
1. Adenomas – Pleomorphic
Monomorphic – Warthin’s tumor
2. Carcinoma:
Low grade
• Acinic cell carcinoma
• Adenoid cystic carcinoma
• Low grade mucoepidermoid carcinoma.
High grade
• Adenocarcinoma
• Squamous cell carcinoma
• High grade mucoepidermoid carcinoma.
3. Nonepithelial tumors:
• Hemangioma
• Lymphangioma
• Neurofibroma.
4. Lymphomas:
• Primary—NHL
• Lymphoma in Sjögren’s syndrome.
5. Secondary:
• Local—tumors of head and neck
• Distant—skin and bronchus.
330
Clinical Surgery Pearls
6. Unclassified tumors
7. Tumor-like lesions:
– Adenomatoid hyperplasia
– Salivary gland cysts.
Q 41. Why pleomorphic adenoma is called mixed
parotid tumor?
It is called mixed parotid tumor because it has got
both epithelial and mesodermal elements.
Q 42. Can pleomorphic adenoma occur bilaterally?
Yes.
Q 43. What are the peculiarities of pleomorphic
adenoma?
• It is considered a benign tumor with long
quiescent periods and short periods of rapid
growth
• Potential for recurrence
• Potential for malignant change.
Features of pleomorphic adenoma
1. The capsule is incomplete and the tumor will have
extensions beyond the capsule
2. Recurrence can occur if tumor excision is not
complete
3. 10% of the tumors are highly cellular and more
liable to recur
4. Tumor contains both epithelial and mesodermal
elements (myoepithelial cells surrounding the
tubules)
5. After surgery for recurrence, radiotherapy is
indicated even though it is benign
6. Benign pleomorphic adenomas metastasize
inexplicably—metastatic pleomorphic adenoma—
it is not malignant
7. It is a tumor readily implanted during removal in
the residual parotid.
Q 44. What are the malignant parotid tumors in
order of frequency?
• Mucoepidermoid carcinoma (most common)
• Malignant mixed tumor
• Acinic cell carcinoma
• Adenoca rcinoma, polymorphous type of
adenocarcinoma (Indian file appearance)
• Adenoid cystic carcinoma—10% (2/3rd in minor
salivary gland)
• Epidermoid carcinoma (squamous cell carcinoma).
Q 45. Which is the carcinoma parotid with worst
prognosis? (PG)
• Carcinoma arising in pleomorphic adenoma
• There is accelerated recurrence rate and high
incidence of metastasis
• Five year survival is less than 40%.
Q 46. What is the type of malignancy in pleomorphic
adenoma? (PG)
1. Carcinoma originating from pleomorphic
adenoma (carcinoma ex-pleomorphic
adenoma) 15 years after the original
swelling–9.5% chance for carcinoma.
Q 47. What are the peculiarities of adenoid cystic
carcinoma?
• Propensity for perineural invasion
• Regional lymph node involvement uncommon
• Distant metastasis occurwithin 5 years(however
they remain asymptomatic for years)
• The malignancy will start as pain in the parotid
region.
Q 48. What is the difference between low grade
and high grade mucoepidermoid carcinoma?
High grade lesions have propensity for both
regional and distant metastasis.
Parotid Swelling
331
Q 49. What is the staging of parotid tumors? (PG)
TNM staging as per AJCC 7th edition is recommended.
TX – Primary tumor cannot be assessed
T0 – No evidence of primary tumor
T1 – Tumor 2 cm or less in greatest dimension
without extraparenchymal extension
T2 – Tumor more than 2 cm but not more than
4 cm in greatest dimension without extraparenchymal extension
T3 – Tumor more than 4 cm and/or tumor having
extraparenchymal extension
T4a – Moderately advanced disease—Tumor
invades skin, mandible, ear canal, and/or
facial nerve
T4b – Very advanced disease—Tumor invades skull
base and/or pterygoid plates and/or encases
carotid artery
Note: N stage is same for all head and neck
malignancies.
Staging
Stage 1 – T1 N0 M0
Stage 2 – T2 N0 M0
Stage 3 – T3 N0 M0
T1 N1 M0
T2 N1 M0
T3 N1 M0
Stage 4A – T4a N0 M0
T4a N1 M0
T1 N2 M0
T2 N2 M0
T3 N2 M0
T4a N2 M0
Stage 4B - T4b
Any T
any N
N3
M0
M0
Stage 4C - Any T Any N M1
Q 50. What is the difference between staging for
major salivary gland tumors and minor salivary
gland tumors? (PG)
The minor salivary gland tumors are located in the
lining of upper aerodigestive tract and they are
staged according to the anatomic site of origin (e.g.
oral cavity, sinuses, etc.).
Q 51. What are the major salivary glands?
They include parotid, submandibular and sublingual glands.
Q 52. What is the regional node spread in parotid
tumor?
Intraglandular node → Periparotid node →
Submandibular node → Upper and mid-jugular
nodes (occasionally to retropharyngeal nodes).
Q 53. What are the causes for bilateral parotid
tumors?
1. Warthin’s tumor
2. Acinic cell carcinoma—2% bilateral.
Q 54. What is the CT sign of inoperability in parotid
carcinoma? (PG)
Involvement of the masseteric space (pre masticator
space) is suggestive of inoperability. It is divided by
zygoma into supratemporal (contains temporalis
muscle) and infra temporal space (contains lateral
and medial pterygoid muscles).
Q 55. What is the CT sign of skull base involvement
in parotid tumor? (PG)
Widening of foramen ovale is suggestive of lower
cranial nerve involvement. Involvement of the
pterygoid plate is another sign.
Q 56. What is the test for temporomandibular joint
involvement? (PG)
a. The little finger is introduced to the external
auditory meatus with the pulp of finger forwards
332
Clinical Surgery Pearls
and simultaneously assess the difference in
range of movement.
b. Inter incisor distance measurement.
Q 57. What is the treatment of pleomorphic
adenoma?
• Superficial parotidectomy is the minimum
surgical procedure
• There is no role for enucleation and excision
(because of the reasons mentioned above)
• Facial nerve should be spared if a plane exists
and when it is not involved
• Facial nerve is scarified only if it is involved or it
is totally encased as in cases of carcinomas.
Q 58. If the facial nerve is involved what is the
treatment option?
The nerve is excised and a nerve graft is done with
Great auricular nerve.
Q 59. What is the management of facial nerve
injury? (PG)
1. Nerve transection is managed by nerve suturing
2. Loss of a segment is managed by cable graft
using great auricular or sural nerve
3. If the proximal end of the nerve is not available
for suturing, hypoglossal nerve transposition or
redirection is done.
Q 60. If facial palsy is identified postoperatively.
What is the management?
1. Give steroids (prednisolone) and wait for
improvement.
2. If there is no improvement re-exploration and
repair is an option.
3. Masseter transfer can be done for the deviation
of the angle of mouth.
4. Temporalis transfer can be done for the
orbicularis oculi function.
Q 61. Is nerve grafting a contraindication for
radiotherapy?
No.
Q 62. What is the timing of radiotherapy? (PG)
3–6 weeks after surgery.
Q 63. What are the indications for radiotherapy?
(PG)
Indications for radiotherapy
(50–70 Gy given in 1.8–2.0 Gy in 5–8 weeks time)
1. T3 and T4 tumors
2. High grade tumors
3. Deep lobe involvement
4. Perineural spread
5. Vascular invasion
6. Multiple lymph node involvement
7. Close margins
Q 64. Is there any indication for chemotherapy?
(PG)
No.
Q 65. What is the surgical treatment of nodes?
Comprehensive neck dissection in the form of
Radical neck dissection is done.
Q 66. What are the important anatomical points
to be remembered in parotid surgery?
1. The gland is situated in the space behind the
ramus of the mandible, below the base of the
skull and in front of mastoid process.
2. Deeply it is applied to the styloid process and
its muscles.
3. The upper pole lies just below the zygomatic
arch and wedged between the meatus and
mandibular joint.
4. Upper pole—The superficial temporal vessels,
the temporal branches of the facial nerve and
Parotid Swelling
333
the auriculotemporal nerve are found entering
or leaving the gland near the upper pole.
5. Lower pole—The cervical branch of the facial
nerve and the two divisions of the posterior
facial vein emerge from its lower pole.
6. Anterior border—Overlies the masseter. The
parotid ducts, the zygomatic, buccal and
mandibular branches of facial nerve emerge
from the anterior border.
7. The external carotid artery, the facial nerve
and the retromandibular vein pass through
the substance of the gland (the external
carotid artery terminates behind the neck of
the mandible by dividing into maxillary and
superficial temporal arteries). Intraparotid
lymph nodes are also seen in the substance of
gland.
8. The facial nerve is seen in the faciovenous
plane of Patey (the nerve is seen superficial to
the posterior facial vein which is formed within
the substance of gland by the continuation
of the superficial temporal vein and emerges
usually into two branches at the lower pole of
the gland).
9. The nerve is dividing the gland into a superficial
lobe and deep lobe. 80% of the gland lies
superficial to the nerve and 20% deep to the nerve.
10. An accessory lobe is present in less than 50%
of the population.
Q 67. What is the incision used for superficial
parotidectomy?
A lazy ‘S’ incision is used—Preauricular—Mastoid
- Cervical incision.
Q 68. What are the essential steps of superficial
parotidectomy?
1. Surgery is done under general anaesthesia with
endotracheal intubation.
2. Lazy ‘s’ incision is used as mentioned above.
3. Infiltration with local anesthetic and adrenalin
for better delineation of the plane.
4. Reflect the skin flaps anteriorly justsuperficial
to the parotid fascia upto the anterior border
of the gland.
5. Back of the parotid gland is identified, and
dissection is carried out to expose the facial
nerve.
6. The sternomastoid is retracted and great
auricular nerve divided in the avascular plane
along the anterior border of the muscle.
7. Identify the posterior belly of digastric.
8. Identify the avascular plane along the anterior
border of cartilaginous and bony external
auditory meatus immediately anterior to the
tragus.
9. Landmarks for identification of facial nerve—
(always identify the trunk of the nerve first
rather than tracing the branches from the
periphery).
a. Conley’s pointer—The inferior portion of the
cartilaginous canal. The facial nerve lies 1cm
deep and inferior to its tip.
b. The upper border of the posterior belly
of digastric muscle—The facial nerve is
usually located immediately superior to it.
c. The stylomastoid artery lies immediately
lateral to the nerve.
10. Identify the two main divisions.
11. Dissect the gland off branches of the facial
nerve.
12. With the exception of buccal branch, all
transected nerves are repaired with cable graft
from great auricular nerve.
13. The desired amount of gland is removed.
14. A suction drain is applied and wound is closed.
334
Clinical Surgery Pearls
Q 69. What is radical parotidectomy?
Radical parotidectomy involves removal of all
parotid gland tissue and elective sectioning of
the facial nerve usually through the main trunk.
The surgery removes ipsilateral masseter muscle
in addition. If there is clinical, radiological, and
cytological evidence of lymph node metastasis a
simultaneous radical neck dissection is carried out.
It is done for:
• High grade malignant tumors
• Squamous cell carcinoma.
Q 70. What are the complications of parotid
surgery?
Complications are
Complications of parotidectomy
1. Seroma
2. Wound infection
3. Permanent facial palsy (transection of the nerve)
4. Temporary facial nerve weakness
5. Facial numbness
6. Permanent numbness of the ear lobe (due to great
auricular nerve transection)
7. Sialocele
8. Frey’s syndrome (Gustatory sweating)
9. Parotid fistula.
Q 71. What is Frey’s syndrome?
This is due to inappropriate regeneration of the
damaged parasympathetic autonomic nerve fibers
to the overlying skin. Salivation resulting from smell
or taste of food, will stimulate the sweat glands of
the over lying skin instead of the parotid. The clinical
features are:
1. Sweating over the region of parotid gland
2. Erythema over the region of parotid gland.
Q 72. What is the clinical test to demonstrate Frey’s
syndrome? (PG)
Starch iodine test—Paint the affected area with
iodine and allow it to dry. Apply dry starch over it.
The starch turns blue on exposure to iodine in the
presence of sweat. The sweating is stimulated after
painting starch.
Q 73. What is the management of Frey’s syndrome? (PG)
Prevention
It can be prevented by placing a barrier between
the skin and parotid bed to prevent inappropriate
regeneration of autonomic nerve fibers. The
following methods are useful—
1. Temporalis fascial flap
2. Sternomastoid muscle flap
3. Artificial membrane between the skin and
parotid bed.
Management of established syndrome:
1. Tympanic neurectomy
2. Injection of botulinum toxin into the affected
skin. (simple and effective method)
3. Antiperspirants—Aluminium chloride.
28 Submandibular Sialadenitis
Case
Case Capsule
A 35-year-old female patient presents with right
submandibular swelling of 4 × 2.5 cm size, firm
in consistency and has pain and increase in size of
the swelling during salivation (eating) for 6 months.
The swelling is bidigitally palpable.
Read the diagnostic algorithm for a swelling.
Checklist for history
1. History of systemic diseases responsible for
sialadenosis like—DM, drugs (antiasthmatic,
guanethidine), endocrine disorders, alcoholism,
pregnancy, bulimia (eating disorders)
2. History of salivary colic
3. Increase in size during salivation
4. History of collagen diseases
5. History of similar swelling on the contralateral side.
Checklist for examination
1. Bidigital palpation with a gloved finger inside the
oral cavity
2. Palpation of the Wharton’s duct for stones in the
floor of the mouth
3. Examine the opening of the duct (sublingual papillae
on the side of the frenulum) for inflammation and
for purulent discharge
4. Look for regional lymph nodes
5. Look for induration/ulceration of the overlying
skin—suggestive of malignancy
6. Look for other salivary glands on both sides.
Contd...
Contd...
Q 1. What is your diagnosis? What is the differential
diagnosis?
• Chronic submandibular sialadenitis (It is bidigitally palpable)
• DD—Submandibular lymph nodes.
336
Clinical Surgery Pearls
Q 8. What are the types of inflammation in the
submandibular gland?
The inflammation of the gland is called sialadenitis.
The types of sialadenitis are:
• Acute
• Chronic
• Acute on chronic.
Q 9. What are the causes for acute sub- mandibular
sialadenitis?
• Viral—mumps
• Bacterial—secondary to obstruction.
Q 10. What are the causes for chronic sialadenitis?
• Obstruction by stone formation, which may be
within the gland (sialolithiasis), within the duct
system
• Trauma to the floor of mouth by denture—subsequent inflammation and stricture of the duct.
Q 11. How many percentage of the submandibular
are radio opaque?
• 80% are radio opaque
• It can be identified in plain radiograph.
Q 12. Which is the commonest site of the submandibular stone—gland or duct?
About 80% of the stones are situated in the sub
mandibular gland.
Q 13. Which type of sialadenitis is more common—
bacterial or viral?
Bacterial.
Q 14. How will you manage this case?
• Investigations for diagnosis
• Investigations for surgery.
Investigations for diagnosis
1. Plain X-ray:Occlusive viewusing dental film.This
will demonstrate radioopaque calculus in the
duct and salivary gland.
Q 2. How to differentiate them?
• If the swelling is bidigitally palpable, it is submandibular salivary gland
• Lymph nodes are not bidigitally palpable.
Q 3. Why salivary gland is palpable bidigitally?
The submandibular salivary gland has a portion
above the myelohyoid muscle (deep lobe) in the
floor of mouth. Therefore, the gland is bidigitally
palpable.
Q 4. What are your points in favor of submandibular salivary gland?
1. Salivary colic—pain induced by salivation as a
result of obstruction to the outflow from the
gland (may be a stone in the duct).
2. Increase in size during salivation (above reason)
3. Decrease in size of the swelling or disappearance
1 to 2 hours after the meal is completed
4. Positive bidigital palpation
5. Solid nature of the swelling.
Q 5. If the swelling is cystic, what are the possibilities?
If it is cystic, one has to rule out a sublingual dermoid.
Q 6. What is the importance of palpating the
Wharton’s duct and its opening (sublingual papillae)?
• Palpation in thegingivolingual sulcus will reveal
stones in the Wharton’s duct.
• Inspection of the sublingual papillae reveals
inflammation and discharge (purulent).
Q 7. Why stones are more common in the
submandibular salivary gland compared to the
parotid?
• The gland and duct system has a shape similar
to the retort.
• The duct is above and the gland is below. The
gland has antigravity drainage.
• The secretion is thicker in the submandibular
salivary gland.
Submandibular Sialadenitis
337
Q19. When the stone is proximal to the crossing
of lingual nerve, what is the management? (PG)
• Intraoral approach is avoided because it leads
to injury to the lingual nerve
• The gland is removed by external approach—
Sialadenectomy along with removal of the
stone from the duct and ligation of the duct.
Q 20. What are the indications for excision of
submandibular salivary gland? (PG)
1. Sialadenitis
2. Stones in the gland
3. Stonesin the duct proximal to the lingual nerve
4. Salivary tumors.
Q 21. What is the difference between sialadenectomy for inflammatory condition and tumor
of the submandibular salivary gland? (PG)
• Intracapsular dissection i s d o n e fo r
inflammatory condition
• Extracapsular dissection with a cuff of normal
tissue around is done for tumor. This may be
combined with suprahyoid neck dissection.
Q 22. What is the incision for sialadenectomy and
what precautions are taken?
It is important to avoid injury to the marginal
mandibular branch of the facial nerve and
therefore the incision is placed 3 to 4 cm below the
lower border of the mandible. A 6 cm long incision
is cited within the skin crease.
Q 23. In which plane you get marginal mandibular
branch of the facial nerve? (PG)
Subplatysmal plane. The skin flaps are raised at
subplatysmal level.
Q 24. Is it necessary to close the platysma at this
situation? (PG)
• Yes. It is sutured with continuous absorbable
suture.
2. USG: Ultrasound is a very useful tool for the
demonstration of stones.
3. FNAC: To rule out a tumor of the salivary gland
and to rule out lymph node.
Investigations for surgery
1. X-ray chest
2. ECG
3. Hemogram
4. Blood sugar
5. Renal status.
Q 15. If no stone is demonstrated and it is found to
be sialadenitis, what is the management?
Bacterial sialadenitis has a poor capacity for
recovery following infection and the gland becomes
chronically inflamed. Therefore, the gland has to be
removed—Sialadenectomy.
Q 16. If stone is demonstrated radiologically, what
is it called?
Sialolithiasis.
Q 17. If the stone is clinically and radiologically
demonstrated in the Wharton’s duct, what is the
management?
If the stone is lying anterior to a point at which
the duct crosses the lingual nerve (second molar
region), the stone can be removed by incising
longitudinally over the duct. This is done under
the local anesthesia.
Q 18. Will you close the duct after removal of the
stone?
• No
• The duct should be left open for free drainage
of saliva
• Suturing will lead to stricture formation and
recurrence of obstructive symptoms.
338
Clinical Surgery Pearls
• Platysma muscle has direct contribution to the
depressor activity of the corner of the mouth.
Q 25. What are the important anatomical
relationships of the submandibular gland?
• Three cranial nerves are at risk:
1. Marginal mandibular branch of the facial nerve
2. The lingual nerve
3. The hypoglossal nerve.
• Two vessels need ligation
1. Anterior facial vein running over the surface
of the gland
2. Facial artery.
• Two Muscles are related to the gland
1. Mylohyoid muscle—around its posterior
border the large superficial lobe becomes
the small deeper lobe
2. The deep part of the gland lies on the
hyoglossus muscle closely related to the
lingual nerve and hypoglossal nerve.
Q 26. What is the anesthesia of choice for
sialadenectomy?
General anesthesia—with endotracheal intubation.
Q 27. What are the peculiarities of the facial artery
in this situation? (PG)
• The course of the artery is variable here
• The artery lies in the groove on the deeper
aspect of the gland
• Sometimes the artery will penetrate the
substance of the gland
• Passes around the gland sometimes
• The artery has to be ligated doubly, superiorly
and inferiorly.
Q 28. What is the landmark for identification of
the deep lobe?
• Posterior border of the myelohyoid
• Once the muscle isretracted forwardsthe deep
lobe can be identified.
Q 29. Is there any attachment of the gland to the
lingual nerve? (PG)
The gland is attached to the lingual nerve through
parasympathetic secretomotor fibers. These
parasympathetic nerve fibers are divided, protecting
the lingual nerve.
Q 30. How do you tackle the submandibular
duct? (PG)
It is identified and ligated as anteriorly as possible.
Q 31. Is there a need for a wound drain after
surgery?
It is better to put a continuous suction drain for
24 hours. There are numerous veins encountered on
the deeper aspect of gland, which are coagulated
or ligated.
Q 32. What are the complications of sialadenectomy?
Complications of submandibular sialadenectomy
1. Injury to the marginal mandibular nerve
2. Hematoma
3. Wound infection
4. Injury to the nerve to mylohyoid producing
submental anesthesia
5. Lingual nerve injury
6. Hypoglossal nerve injury.
Q 33. What are the clinical features of malignancy
in the gland?
Signs of malignancy in submandibular salivary gland
• Rapid increase in size
• Induration
• Ulceration of the overlying skin
• Cervical node enlargement.
Submandibular Sialadenitis
339
Q 34. What is the incidence of malignancy in submandibular salivary gland?
About 50% are malignant in contrast to the parotid
where only 20% are malignant. The chances of
malignancy increase from parotid to submandibular
to sublingual and minor salivary glands.
Q 35. What are the investigations for diagnosis in
suspected tumors of the submandibular salivary
gland?
• FNAC—It is the investigation of choice.
• CT/MRI—If required to know the nature of
surrounding invasion.
Q 36. Is there any contraindication for open
biopsy?
Open biopsy is contraindicated because of the
tumor seedling.
Q 37. What is “Stafne Bone Cyst”? (PG)
It is an ectopic lobe of the submandibular salivary
gland presenting as asymptomatic radiolucency
of the angle of the mandible, below the inferior
dental neurovascular bundle. Edward C Stafne
a dental surgeon from Mayo clinic described
this condition. No treatment is required for this
condition.
29 Ranula, Plunging Ranula,
Sublingual Dermoid and Mucous Cyst
Case
Case Capsule
A 20-year-old male patient presents with a bluish
tinged spherical cystic swelling 5 × 3 cm size in the
floor of the mouth on one side of frenulum lingulae
(sublingually). It is translucent. Externally there is
no visible swelling.
Read the diagnostic algorithm for a neck swelling.
Checklist for history
1. History of trauma
2. History of long duration.
Checklist for examination
1. Look for color—blue or opaque white
2. Decide whether it is solid or cystic
3. Decide whether the swelling is purely intraoral or
it is extending down to the neck
4. Look for swelling beneath the chin
5. Bimanual palpation, if there is swelling beneath the
chin decide whether the intraoral part is continuous
with the swelling beneath the chin
6. Decide whether it is midline or lateral
7. Look for the submandibular duct traversing the
dome of the cyst in the floor of the mouth
8. Look for translucency.
Q 1. What is your diagnosis?
Ranula.
Q 2. What are the diagnostic points in favor of
ranula?
1. Tense cystic swelling in the floor of the mouth
2. It is blue in color
3. It is translucent
4. It is situated to one side of frenumlingulae
5. The submandibular duct can be seen traversing the
dome of the cyst.
Ranula, Plunging Ranula, Sublingual Dermoid and Mucous Cyst
341
Q 11. What are the complications of ranula?
1. Infection
2. Mechanical interference with speech
3. Difficulty in eating.
Q 12. What is the surgical treatment of ranula?
Excision of the cyst and the affected sublingual
gland.
Q 13. What is the approach for surgery?
Intraoral approach is preferred.
Q 14. Why not incision and drainage of the cyst?
It is not recommended, because it will result in
recurrence of the cyst.
Q 15. What is the surgical approach for plunging
ranula?
Excision is performed via cervical approach.
Q 16. What is the incision for cervical approach?
• The incision is similar to submandibular
sialadenectomy
• The cyst together with submandibular and
sublingual salivary glands are excised.
Q 17. What is mucous cyst?
It is an extravasation/retention cyst in relation to
minor salivary gland.
Q 18. What is the commonest site for mucous cyst?
Lower lip.
Q 19. What is the cause for mucous cyst?
It is as a result of trauma to the overlying mucosa.
Q 20. What is the transillumination finding in
mucous cyst?
It may or may not be translucent.
Q 21. What is the surgical treatment?
• Formal surgical excision is required along with
the affected minor salivary gland under local
anaesthesia.
• Some may resolve spontaneously.
Q 3. What is the most important differential
diagnosis?
Sublingual dermoid cyst.
Q 4. What are the clinical points against sublingual
dermoid in this case?
• Translucencyisapointagainstsublingualdermoid
• Sublingualdermoidmaybeplacedinthemid-line
unlike ranula which is to one side of the frenum
• Sublingualdermoidisnotblue incolor. Itiswhite
and opaque.
Q 5. What is ranula?
It is a mucous extravasation cyst arising from the
sublingual salivary gland.
Q 6. Why it is called ranula?
It is called ranula because of its resemblance to
frog’s belly.
Q 7. Who gave this name?
Hippocrates.
Q 8. What is plunging ranula?
Here the cyst penetrates the mylohyoid diaphragm
to enter the neck. Therefore, there will be a swelling
in the floor of the mouth along with a swelling in the
neck, resembling a dumbbell. This retention cyst
may be arising from sublingual or submandibular
salivary gland. The neck swelling may be in the
submental or submandibular region of the neck.
Q 9. What is the clinical test for plunging ranula?
Here one should elicit the Bidigital palpation – with
index finger of one hand in the mouth and fingers of
the other hand exerting upward pressure externally
from the below the lower jaw over the neck swelling.
In case of plunging ranula it will be positive.
Q 10. How to confirm the diagnosis of ranula?
1. Ultrasound examination
2. MRI (for plunging ranula)
3. Plain X-ray to rule out stones.
342
Clinical Surgery Pearls
1. Color-opaque white cyst in contrast to ranula
2. Content—sebaceous material
3. May present as swelling in the floor of the mouth
or swelling beneath the chin
4. No attachment to the covering mucosa or skin
5. Do not transilluminate.
Q 28. What are the two most important examinations
in suspected sublingual dermoid cyst?
1. Examination of the neck to rule out an extension
beneath the chin or laterally in the submandibular
region in the case of the lateral variety
2. Bimanual palpation—positive bimanual palpation
suggests extension beneath the mylohyoid.
Q 29. What are the other differential diagnoses of
a swelling beneath the chin?
• Thyroglossal cyst
• Subhyoid bursa.
Q 30. What is the incision for excision of a median
sublingual dermoid?
1. If there is extension beneath the chin, consider
external incision in the submental region.
Division of mylohyoid may be required. The cyst
is then enucleated.
2. If there is no extension beneath the chin, intraoral
approach will suffice for the enucleation.
Q 31. How a sublingual dermoid cyst is formed?
During the process of fusion of the facial processes,
a piece of the skin may get trapped deep in the
midline just behind the jaw and later form the
dermoid cyst. The cyst may be midline or lateral.
Q 32. What is the age group affected?
It is 10–25 years (Both sexes equally affected).
Q 33. What are the complications of sublingual
dermoid?
1. Infection (painful)
2. Interfere with eating (when it is big)
3. Interfere with speech.
Q 22. How many minor salivary glands are there
in the oral cavity?
Around 450 (They contribute 10% of the salivary
volume).
Q 23. What is the distribution of minor salivary gland?
Sites of minor salivary glands
• Cheek
• Palate
• Floor of the mouth
• Lips
• Retromolar area
• Upper aerodigestive tract:
– Oropharynx
– Larynx
– Trachea
– Sinuses.
Q 24. Is it possible to get a tumor in the sublingual
salivary gland?
Yes. They are extremely rare. They present as firm or
hard painless swelling in the floor of mouth. 95%
are malignant.
Q 25. What is the surgical management of this
malignancy?
Wide excision along with overlying mucosa
combined with suprahyoid neck dissection.
Q 26. What are the types of sublingual dermoid?
There are two types:
1. Median—in the midline
2. Lateral:
• It may be situated above the mylohyoid—
supramylohyoid variety (floor of the mouth)
• It may be below the mylohyoid—inframylohyoid variety (swelling beneath the
chin)—double chin appearance.
Q 27. What are the diagnostic points in favor of
sublingual dermoid?
Diagnostic points for sublingual dermoid
30 Thyroglossal Cyst, Lingual Thyroid,
Ectopic Thyroid Subhyoid Bursa and
Carcinoma Arising in Thyroglossal Cyst
Case
Case Capsule
A 15-year-old girl presenting with a spherical cystic
swelling in front of the neck beneath the hyoid
bone of 1.5 cm diameter. There is movement with
protrusion of the tongue. The swelling is moving
up and down with deglutition.
Read the diagnostic algorithm for a swelling.
Checklist for history
• Family history of goiter
• History of radiation to the neck
• History of dysphagia
• History of dysphonia
• History of dyspnea
• History of pain over the swelling
• History of rapid increase in size of the swelling
• History of discharge from the swelling
• History of operations for the swelling.
Checklist for examination
1. Elicit fluctuation by Paget’s method : it may appear
as firm or tense cystic. Some cysts are too small to
fluctuate
2. Look for transillumination
3. Look for movement with protrusion of the tongue
and movement with deglutition
4. Always examine the oral cavity : Base of the tongue
for ectopic thyroid/lingual thyroid
5. Always palpate for the presence of normal thyroid
and cervical ectopic thyroid
6. Look for regional lymph node enlargement.
Contd...
Contd...
Q 1. What is the most probable diagnosis in this
case?
Thyroglossal cyst.
344
Clinical Surgery Pearls
7. Dermoid cyst
8. Collar—stud abscess in connection with a lymph
node
9. Ectopic thyroid
10. Sebaceous cyst
11. Lipoma
Q 5. What are the other midline swellings in front
of the neck?
Midline swellings in front of the neck (in addition to
the differential diagnoses given above).
• Ludwig’s angina in the upper part
• Retrosternal and plunging goitre in the lower part
• Swellingsfrom the Space of Burns in the lower part
(lymph node and lipoma)
• Dermoid cyst in the lower part
• Thymic swellings
• Aneurysm of innominate artery.
Q 6. What are the causes for enlarged submental
lymph node?
1. Inflammatory—Specific like tuberculosis
—Nonspecific
2. Neoplastic—Primary—Hodgkin’s and NHL
— Secondary from carcinoma of
lower lip, tip of tongue, floor of
mouth.
Q 7. What is sublingual dermoid?
It is a sequestration dermoid cyst secondary to
sequestration of surface ectoderm at the site
of fusion of mandibular arches. The swelling is
situated in the midline in the floor of the mouth.
The lateral variety of sublingual dermoid arises from
second branchial cleft. It is soft, cystic and lined
by squamous epithelium. It contains sebaceous
material and therefore not translucent.
Q 2. What are the diagnostic points in favor of
thyroglossal cyst?
1. The most important sign of thyroglossal cyst is
the upward movement of the swelling when
the tongue is protruded. This is because of its
connection via the thyroglossal duct with the base
of the tongue. It will also move up and down with
deglutition. In addition, there will be horizontal
movement but no vertical movement.
2. The presence of a small cystic swelling in the
midline of the neck (seldom large enough to
exhibit fluctuation).
3. May or may not be translucent (when the
content is thick as a result of past infection, the
cysts do not transilluminate).
Q 3. How will you demonstrate the upward
movement during protrusion of the tongue?
a. Request the patient to open the mouth
b. Grasp the swelling between the finger and
thumb
c. Instruct the patient to put out the tongue
d. The positive test will give an unmistakable
upward tug (certain amount of movement will be
there in this region for all swellings). The mouth
must be open when the tug is appreciated.
e. The patient may be instructed to put the tongue
in and out again if required.
Q 4. What are the differential diagnoses?
Differential diagnoses of thyroglossal cyst
1. Median sublingual dermoid
2. Enlarged submental lymph node
3. Subhyoid bursa
4. Enlarged Delphian node
5. Solitary thyroid nodule
6. Thyroid cyst
Contd...
Contd...
Thyroglossal Cyst, Lingual Thyroid, Ectopic Thyroid, Subhyoid Bursa
345
Q 11. Why there is more chance of infection in
thyroglossal cyst?
The wall of the cyst contains lymphatic tissue and
with attacks of respiratory infection, the cyst will
get infected.
Q 12. Once an abscess is formed, what is the
treatment?
Incisionanddrainageisthetreatment.Formalexcision
ofthetractisdone(Sistrunk’soperation)after 6 weeks.
Q 13. What is the classification of cyst in general?
Cysts are classified into congenital cyst and acquired
cyst.
Co n g e n i t a l c ys t s —
examples
Acquired cysts—examples
• Thyroglossal cyst • Sebaceous cyst
• Branchial cyst • Mucous cyst of mouth
(retention)
• Urachal cyst • Cystadenoma (neoplastic)
• Hydatid of Morgagni • Teratoma (neoplastic)
• Dermoid cyst • Hydatid (parasitic)
• Enterogenous cyst •Implantation dermoid
• Cystic hygroma • Traumatic cyst
(Hematoma)
• Lymphatic cyst of
greater omentum
Q 14. What are the complications of cysts in general?
Complications of cysts anywhere
1. Hemorrhage—breathingdifficultiesinthyroglossal cyst
2. Infection—pain
3. Torsion—present as acute abdomen in ovarian cyst
4. Pressure effects on adjacent structures—abdominal
fullness in abdomen
5. Obstruction to pelvic veins—manifest as varicose
veins in the case of large ovarian cyst
6. Calcification.
Q 8. How will you differentiate solitary thyroid
nodule from thyroglossal cyst?
The sign of upward tug during protrusion of
the tongue is absent in the case of the thyroid
nodule.
Q 9. What are the positions of the thyroglossal
cyst?
1. Suprahyoid: Here the swelling is situated
immediately above the hyoid bone and the
differential diagnosis is median sublingual
dermoid.
2. Subhyoid: It is the commonest site of
thyroglossal cyst.
3. At the level of the thyroid cartilage (second
commonest position): At this level the cyst is
usually to one side of the midline (left side)
because the thyroid cartilage is shaped like the
prow of a ship and may be because of the levator
glandulae thyroide muscle
4. At the level of the cricoid cartilage: The
thyroglossal cyst at this level is less common
and the differential diagnosis of thyroid nodule
comes here.
5. Beneath the foramen cecum
6. In the floor of the mouth.
Q 10. What are the complications of thyroglossal
cyst?
1. Infection: The overlying skin will be hot and red.
2. Thyroglossal fistula (Result from bursting or
incision for infection): The thyroglossal fistula
is always acquired in contra-distinction to the
Branchial fistula which is always congenital.
3. Rarely carcinoma: Malignant potential of
dysgenetic thyroid tissue causes papillary
thyroid cancer in 1% of cases (Papillary carcinoma
develops more frequently in ectopics than
normal thyroid).
346
Clinical Surgery Pearls
Q 15. What is the typical appearance of a
thyroglossal fistula?
The skin surrounding the opening has a peculiar
crescentic appearance due to the uneven rate of
growth of the thyroglossal tract (semilunar sign).
Q 16. What is the lining of thyroglossal fistula?
It is lined by columnar epithelium.
Q 17. What is the nature of discharge of
thyroglossal fistula?
Mucus (because of the lining)—in tuberculous
sinus, the discharge will be purulent.
Q 18. What is the differential diagnosis of
thyroglossal fistula?
Tuberculous sinus—especially when the fistula
is situated low in the neck (the discharge will be
purulent).
Q 19. What is the commonest site for thyroglossal
fistula?
1. Just below the hyoid bone
2. Thyroglossal fistulas originating in the infancy
tend to be situated lower in the neck.
Q 20. What is the development of thyroid?
The thyroid gland is endodermal in origin and
develops from the median bud of the pharynx
between 1st and 2nd pharyngeal pouch and
descends along the midline of the neck to lie
anterior to the second, third and fourth tracheal
rings. Each thyroid lobe amalgamates with the
ultimobranchial body (neuroectodermalin origin)
arising as a diverticulum of the fourth pharyngeal
pouch on each side. The parafollicular cells (C cells)
from the neural crest reach the thyroid via the
ultimobranchial body. The line of descent of thyroid
is called the thyroglossal tract. The thyroglossal
tract extends from the foramen cecum (vestigial
remnant of the duct) of the tongue to the isthmus
ofthe thyroidgland.Usually the tractwill completely
atrophy by 5th week. If any portion of this tract
remains patent, it can form a cyst. Theoretically,
thyroglossal cyst can occur anywhere between the
bases of the tongue and the isthmus of the thyroid
gland (between the chin and second tracheal ring).
The tract descends through the 2nd branchial arch
anlage, i.e. hyoid bone prior to fusion in the midline.
Q 21. What is the treatment for thyroglossal cyst?
Sistrunk’s operation.
Q 22. What is Sistrunk’s operation?
It consists of removal of the thyroglossal cyst
along with the entire thyroglossal tract up to the
foramen cecum. The central part of the body of the
hyoid bone (1cm) is excised during the process of
excision of the tract (In majority of the cases the
tract will be going behind the body of the hyoid
bone). It is important to core out the entire tract
in the floor of the mouth up to the foramen cecum.
Q 23. If the thyroglossal cyst is low down, can you
core out the entire tract through a single incision?
No. Initially a skin line horizontal incision is put over
the cyst and the tract is dissected up to the hyoid
bone. At this level, another skin line incision may
be put so that the entire tract can be cored out up to
the base of the tongue. The same technique is used
for the surgical treatment of thyroglossal fistula.
Q 24. What are the clinical features of carcinoma
arising in the thyroglossal cyst? (PG)
Features of malignancy in thyroglossal cyst
• Recent rapid increase in size of the cyst
• Hard consistency
• Fixity
• Irregularity
• Presence of enlarged lymph node.
Thyroglossal Cyst, Lingual Thyroid, Ectopic Thyroid, Subhyoid Bursa
347
Q 25. What is the treatment of carcinoma arising
in the thyroglossal cyst? (PG)
• The usual surgery performed is Sistrunk’s
operation if the thyroid is found to be normal
(Routine thyroidectomy is not recommended
in all patients with carcinoma in thyroglossal
cyst). The indications for thyroidectomy are:
1. Nodular thyroid with cold nodule
2. Presence of enlarged neck nodes
3. History of irradiation to the neck.
Following thyroidectomy radioiodine ablation
is recommended:
• Thyroid suppression is recommended for
all patients with papillary carcinoma of the
thyroglossal duct cyst regardless of the status
of thyroid
• Long-term follow-up is mandatory.
Q 26. What is ectopic thyroid?
Presence of residual thyroid tissue along the course
of the thyroglossal tract is called ectopic thyroid.
The ectopic thyroid may be:
1. Lingual
2. Cervical
3. The whole gland may be ectopic.
Q 27. What is the manifestation of lingual thyroid?
• It will present as a swelling at the back of the
tongue in the region of foramen cecum.
• Always palpate the neck and make sure that the
normal thyroid is present (If bare tracheal rings
are palpated in the midline, one should suspect
absence of thyroid in the normal position. It may also
be due to absence of the isthmus of the thyroid).
The symptoms of lingual thyroid
• Dysphagia
• Dysphonia
• Dyspnea
• Hemorrhage
• Pain
• Carcinoma (develops more frequently in ectopic
thyroid tissue than in normal thyroid gland).
Q 28. What are the differential diagnoses of lingual
thyroid? (PG)
Differential diagnoses of lingual thyroid
1. Hypertrophied lingual tonsil
2. Carcinoma of the tongue
3. Fibroma
4. Angioma
5. Sarcoma
6. Ranula.
Q 29. What is athyreosis?
Athyreosis
• Absence of palpable lateral lobes
• Absence of isthmus
• Hypothyroidism.
Q 30. What is the investigation of the choice in
lingual thyroid?
1. FNAC
2. Radioiodine scintiscan—to find out whether it
is the only functioning thyroid and to find out
the presence of normal thyroid.
Q 31. What is the treatment of choice in lingual
thyroid?
The treatment options are:
1. Thyroid suppression with thyroid hormone—it
should get smaller.
Contd... OR
Contd...
348
Clinical Surgery Pearls
2. Ablation with radioiodine.
OR
3. Excision if it is causing symptoms and replacement therapy with thyroid hormone (if it is the
only functioning thyroid).
Q 32. What is median ectopic thyroid?
Itforms a swelling in the upper part ofthe mid- line
of the neck and it is one of the differential diagnoses
of thyroglossal cyst. It may be the only functioning
thyroid and therefore rule out presence of normal
thyroid before excision.
Q 33. What is lateral aberrant thyroid?
This is a misnomer. Any normal tissue found laterally
separate from the thyroid gland must be considered
as lymph node metastasis from occult papillary
thyroid cancer and treated as such.
Q 34. What is struma ovarii?
This is nothing but ovarian teratoma with thyroid
tissue. Rarely, it can produce hyperthyroidism or
neoplastic change.
Q 35. Can agenesis of thyroid occur?
Yes. Usually agenesis is seen on left side.
Q 36. How to make a diagnosis of subhyoid bursa?
1. Clinically
• The swelling islocated below the hyoid bone
• In front of the thyrohyoid membrane
• Transversely oval swelling
• Moves up with deglutition
• Soft and cystic
• Not translucent (turbid fluid).
2. FNAC.
Q 37. What is the treatment of subhyoid bursa?
Excision.
31 Branchial Cyst, Branchial
Fistula, Cystic Hygroma
Case
Case Capsule
A 25-year-old male presenting with a cystic swelling
of about 5 × 3 cm size at the anterior border of the
sternomastoid muscle at the junction of upper and
middle third on the right side of the neck. It is cystic
and fluctuant. There is no transillumination.
Read the diagnostic algorithm for a swelling.
Checklist for history
• Present from birth or not
• History of intermittent swelling
• History of attacks of inflammation
• The nature of discharge
• History of previous surgery.
Checklist for examination
1. Assess the plane of the swelling
2. Look for fixity to surrounding structures
3. Look for fluctuation
4. Look for translucency
5. Look for compressibility
6. Examine the contralateral side for similar swellings
7. Look for other arch problemslike accessory tragi
and periauricular sinuses and cysts
8. Look for lymph nodes
9. Look for the nature of discharge from fistula
10. Rule out pharyngeal communication.
350
Clinical Surgery Pearls
The second arch over grows and joins with the
5th arch producing the buried space lined by
squamous epithelium. This space is called cervical
sinus of His. Normally, it disappears entirely.
Should a part of the space persist, it will form a
branchial cyst (Fig. 31.1).
Q 5. How many branchial arches are there?
Six branchial arches with five pharyngeal pouches
internally and five branchial clefts externally. The
branchial pouches are lined by endoderm and the
clefts are lined by ectoderm.
Q 6. What is the supporting evidence for the
epithelial inclusion in the lymph node?
Most branchial cysts have lymphoid tissue in their
walls.
Q 7. What is the most common age group affected?
Even though itis a congenital abnormality the most
common age group is 3rd decade (suggesting a
different pathogenesis).
Q 8. How to explain the late appearance of the
cyst if it is congenital?
Initially the cyst is an empty sac of embryological
tissue. The epithelial debris accumulates for
Q 1. What is the probable diagnosis in this case?
Branchial cyst.
Q 2. What are the characteristic features of
branchial cyst?
Clinical features of branchial cyst
1. It is situated at the junction of upper and middle
third of the anterior border of the sternomastoid
2. 2/3rd of the swelling is anterior to the sternomastoid
and 1/3rd deep to the sternomastoid
3. The cyst is a loose cyst (It is not a tense cyst)—the
consistency is compared to that of a half filled hot
water bag
4. May or may not be translucent.
Q 3. What are the theories of origin of branchial
cyst?
There are two theories:
1. A rising from the cervical sinus of His
(Developmental origin)
2. Epithelial inclusion within a lymph node.
Q 4. What is cervical sinus of His?
In the 3rd week of embryonic life, a series of
mesodermal condensations known as branchial
arches appearin the walls ofthe primitive pharynx.
Fig. 31.1: Branchial arch pathology
Branchial Cyst, Branchial Fistula, Cystic Hygroma
351
Q 13. What is the lining of branchial fistula?
The fistula is lined with squamous epithelium up
to the partition (cleft membrane). Internal to the
partition it is lined with ciliated columnar epithelium.
Q 14. What is the course of the branchial fistula?
It is below the 2nd arch structures and above the
3rd arch structures.
Q 15. What is the nerve and artery of 2nd and
3rd arches?
• The nerve of 2nd arch is facial nerve
• The arteryofthe 2ndarchis external carotidartery
• The nerve of 3rd arch is 9th
• The artery of 3rd arch is internal carotid.
Q 16. What is the time of clinical presentation of
branchial fistula?
Theopeningispresentatbirth(Itisalwayscongenital).
Q 17. What is the clinical feature other than the
opening?
Mucoid discharge isnoticedinchildrenafterahotbath.
Q 18. What is the exact course of the branchial
fistula?
The external opening is usually seen in relation to
the lower 3rd ofthe anteriorborder ofsternomastoid
muscle. From its opening on the skin it passes
subcutaneously to the level of the upper border of
the thyroid cartilage where it pierces the deep fascia.
The fistula then passes beneath the posterior belly
of the digastric muscle and the stylohyoid muscle,
crosses the hypoglossal nerve and internal jugular
vein, to traverse the fork of the carotid bifurcation.
Here the external carotid artery issuperficial and the
internal carotid deep to the tract. It then crosses the
glossopharyngeal nerve and the stylopharyngeus
muscle to pierce the superior constrictor and to open
on the posterior pillar of the fauces behindthetonsil.
number of years with super added infection
making the appearance of the cyst.
Q 9. Why branchial cysts do not transilluminate?
The cyst contains yellow fluid with cholesterol
crystals and epithelial debris, therefore, it will not
transilluminate.
Q 10. How a branchial fistula is formed?
Should the second arch fail to fuse with the 5th
arch, an opening will be found on the neck at birth
along the anterior border of the sternomastoid
muscle atthe junctionofthemiddle andlowerthird.
Q 11. Is there any communication of the fistula
to the pharynx?
Usually, they are separated from the pharynx by
a septum, which represents the remains of the
cleft membrane (ending blindly on the lateral
pharyngeal wall like a sinus). If the fistulous tract
is complete, it will open just behind the tonsil
on the affected side (On the anterior aspect of
the posterior faucial pillar). There may be small
amount of mucus or mucopurulent discharge
coming out through the fistulous opening. If the
tract is communicating into the oral cavity the
liquids taken during meals will come out through
the fistulous opening.
Q 12. What is the lining of the branchial cyst?
1. Branchial cyst—stratified squamous epithelium
2. Branchial fistula—stratified squamous or
pseudosquamous epithelium externally and
non-ciliated columnar epithelium inside
3. Thyroglossal cyst—pseudostratified ciliated
columnar epithelium
4. Thyroglossal fistula—columnar epithelium.
352
Clinical Surgery Pearls
Q 19. Can the fistula be bilateral?
Yes. In 30% of cases.
Q 20. Can the branchial cyst be bilateral?
Yes. In 2% of cases it is bilateral.
Q 21. On which side the branchial cyst is more
commonly seen?
It is seen 60% on left side.
Q 22. Which sex is more affected?
Males are more affected – 60%.
Q 23. Can the cyst be intermittent?
Yes. In 20% of the patients, the cyst will be
intermittent.
Q 24. If the cyst is disappearing at the time of
surgery? What is the course of action?
There is no place for exploration in this situation.
The cyst may not be found at exploration. Partial
excision will lead on to sinus formation.
Q 25. What are the other associated congenital
anomalies?
Associated anomalies in branchial cyst
• Preauricular sinuses
• Periauricular cysts
• Accessory tragi
• Subcutaneous cartilaginous nodules.
Q 26. The branchial fistula is congenital or
acquired?
• The branchial fistula is always congenital
(the position of the cyst is in the upper part
and the fistula is in the lower part due to the
developmental reasons already mentioned)
• The thyroglossal fistula is always acquired.
Q 27. Can similar swellings occur in relation to
other cleft apparatus?
Yes. It can occur in relation to first branchial cleft.
Q 28. What is bronchogenic carcinoma?
Itis controversialwhetherthis entity is amalignancy
in branchial cyst or it is a cystic degeneration in a
lymph node containing depositfrom squamous cell
carcinoma. The latter is more possible. The primary
growth may not be apparent and it may be situated
in the nasopharynx,tonsil, base oftongue, pyriform
fossaorsupraglottic larynx.Therefore, itisimportant
to rule out an occult primary in such conditions.
Q 29. What are the other cysts having cholesterol
crystals?
• Hydrocele
• Dental cyst
• Dentigerous cyst.
Q 30. What are the most important differential
diagnoses for a branchial cyst?
Othercysticswellingsonthelateralsideofthenecklike.
Cystic swellings on the side of the neck
• Branchial cyst
• Cystic hygroma (Lymphangioma)
• Hemangioma
• Cold abscess
• Dermoid cyst
• Laryngocele
• Pharyngocele
• Sebaceous cyst.
In addition, the following solid swellings form
differential diagnoses for branchial cyst.
Solid swellings on the side of the neck
1. Reactive lymphadenitis
2. Lipoma
3. Neurofibroma
4. Chemodectoma (potato tumor)
5. Paraganglioma
6. Lymphoma
7. Metastatic carcinoma in node from thyroid
8. Tuberculous lymph node.
Branchial Cyst, Branchial Fistula, Cystic Hygroma
353
Q 31. How to differentiate clinically cystic hygroma
from branchial cyst?
• Cystic hygroma is brilliantly translucent
• Cystic hygroma is partially compressible
• Usually seen in the neonate and early infancy
• Increase in size when the child cries or coughs.
Q 32. Why cystic hygroma is brilliantly translucent?
• The cysts are filled with clear fluid
• Linedby single layerof epithelium(withamosaic
appearance).
Q 33. How a cystic hygroma is formed?
Cystic hygroma developsfrom the primitive lymph
sac called jugular lymph sac (they are situated in
the neckbetween the jugular and subclavian veins).
Sequestration of a portion of jugularlymph sac will
result in the formation of cystic hygroma.
Q 34. What are the other situations where you get
cystic hygroma?
Sites for cystic hygroma
• Axilla
• Groin
• Mediastinum
• Cheek
• May involve salivary glands
• Tongue
• Floor of the mouth.
Q 35. What are the complications of cystic hygroma?
• Infection
• Increase in size and respiratory embarrassment
• Obstructed labor during delivery.
Q 36. What are the treatment options for cystic
hygroma?
1. Meticulous conservative neck dissection with
excision of all lymphatic tissue
2. Injection ofsclerosing agents(Picibanil orOK432
is a recent sclerosant).
Q 37. What are the problems of sclerotherapy for
cystic hygroma?
• Extracystic injection can produce inflammation
of adjacent tissue
• Usually they are multicystic and extensive and
therefore difficult to eradicate
• Recurrence is common after sclerotherapy.
Q 38. What are the investigations in branchial cyst?
• Ultrasound
• FNAC
• Aspiration and examination under microscope
for cholesterol crystals
• CT/MRI if paraganglioma is suspected.
Q 39. What are the complications?
• Infection
• Abscess formation.
Q 40. What will happen if formal excision is carried
out during the stage of inflammation?
• It will result in fistula formation
• Do the surgery onlywhen the lesion is quiescent
(2 to 3 months later).
Q 41. What is the treatment of branchial cyst?
Excision of the cyst under general anesthesia isthe
treatment. If the cyst is large and tense, it may be
decompressed with a large bore needle to facilitate
the dissection.
Q 42. What are the structures to be taken care of
during surgery?
• The cystmaybepassingbackwards andupwards
through the carotid fork and reach as far as the
pharyngeal constrictors.
• It passes superficial to the hypoglossal and
glossopharyngeal nerves and deep to the
posterior belly of the digastric.
• The spinal accessory nerve must be identified
and protected.
354
Clinical Surgery Pearls
Q 43. What is the most important investigation
for branchial fistula?
Fistulogram (thiswillprovideinformationregarding
the nature, whether it issinus or fistula). The entire
tract must be removed to prevent recurrence.
Q 44. What is the covering of the fistula external
to the epithelial lining?
Muscle fibers and lymphoid tissue.
Q 45. What is the surgical treatment for branchial
fistula?
Complete excision of the tract.
Q 46. What is the incision used for excision of
the tract?
Elliptical and Step ladder
1. Initially an elliptical incision around the opening
and the tract is dissected through the deep
cervical fascia.
2. Step ladder incisions later on and tract is
followed towards the pharyngeal wall (any
mucosal breach of the pharynx is repaired).
Q 47. If the fistula is asymptomatic, is there any
need for surgical treatment?
• There is no need to treat asymptomatic fistula
other than cosmetic reasons
• Once there is discharge, it should be excised
because patient is likely to get repeated
infections.
32 Soft Tissue Sarcoma
Case
Case Capsule
A 50-year-old male patient presents with swelling
in front and lateral part of right thigh of about
15 × 10 cm size, hard in consistency, deep to deep
fascia with restricted mobility. The regional nodes
are not palpable. There is no distal neurovascular
deficit. The movements of right knee joint are
normal. The flexion of right hip joint is restricted
due to the size of the swelling. Abdominal and chest
examination are normal.
Read the diagnostic algorithm for a swelling.
Checklist for history
• History of radiation
• Historyofchemicalexposure—Arsenic,vinylchloride
• History of lymphedema
• History of familial lymphedema
• History of neurofibromatosis
• History of retinoblastoma
• History of familial polyposis coli.
Checklist for examination
1. Look for other swellings
2. Look for pigmented lesions in the body
3. Local rise of temperature and tenderness
4. Look for dilated veins over the swelling
5. Assessment of the plane of the swelling
6. Involvement of muscle groups
7. Involvement of neurovascular bundle with distal
neurovascular deficit—check for distal pulsations
and sensations
8. Look for movement of the swellings (whether it
is fixed to the bone or not)
9. Check for movement of the joints both proximal
and distal
10. Look for wasting of muscles
11. Look for regional nodes
12. Examine the chest for metastasis.
Contd...
Contd...
356
Clinical Surgery Pearls
undifferentiated mesenchymal stem cells that
may be found virtually anywhere. This will explain
the origin of a sarcoma from smooth muscle where
anatomically smooth muscle is not present.
Q 3 .What are the differential diagnoses?
Benign soft tissue swelling such as:
• Lipoma
• Myositis ossificans
Q 1. What is the most probable diagnosis in this case?
Soft tissue sarcoma (STS).
Q 2. What is soft tissue sarcoma?
They are malignant tumors that arise from skeletal
and extraskeletal connective tissue, mesenchymal
cells including adipose tissue, bone, cartilage,
smooth muscle and skeletal muscle. As per the
new definition they are thought to arise from
Soft Tissue Sarcoma
357
for retroperitoneum. This is followed by carefully
planned biopsy—Core biopsy or incisional biopsy.
The entry point of the core needle biopsy must be
carefully placed such that it does not compromise
subsequent radical excision. The incision is placed
along the future resection axis, i.e. longitudinal for
extremity soft tissue sarcoma.
Q 7. Why not FNAC?
There is no role for FNAC in a suspected case of
soft tissue sarcoma. By FNAC you get a report of
spindle cell neoplasm which is not going to guide
the further management. The core biopsy will give
the following:
1. Histopathological confirmation
2. Evaluate the grade
3. Identify the prognostic factors.
The only role of FNAC is for the confirmation of
recurrence rather than the primary diagnosis.
Q 8. If the core biopsy is negative, is there any
role for biopsy and what are the precautions to
be taken?
Yes.Acarefully planned incision biopsy is donewith
following precautions:
Precautions for biopsy
1. The incision must always be vertical and not
horizontal centerd over the mass in its most
superficial location (An elliptical incision to include
the scar of the biopsy is used for formal wide
excision later on). No tissue flaps are raised
2. Hemostasisis very important at the time of biopsy
(hematoma can distort anatomy)
3. Use drains only if itis absolutely necessary. (Should
not be used lateral to the vertical incision). The drain
site should be as close to the incision as possible
4. If the swelling is less than 3 cm size, excisional
biopsy is recommended.
• Angiomyolipoma
• Hematoma
• Angiomyxoma.
Q 4. What are the etiological factors for soft tissue
sarcoma?
1. Genetic predisposition
• Neurofibromatosis – Von Recklinghausen’s
disease
• Li-Fraumeni syndrome
• Retinoblastoma
• Gardner’s syndrome (familial adenomatous
polyposis)
2. Radiation exposure
3. Lymphedema
• Postsurgical
• Postirradiation
• Parasitic infection (filariasis)
4. Trauma
5. Oncogene activation
• MDM2, C-erB2
, C-KIT
6. Chemical
• 2, 3, 7, 8 – Tetrachlorodibenzodioxin
• Polyvinyl chloride
• Chlorophenols
• Phenoxy acetic acid.
Q 5. What is the pathogenesis of soft tissue
sarcoma (STS)?
• Specific genetic alterations—fusion genes due
to reciprocal translocations and specific point
mutations
• Nonspecific genetic alterations—genetic losses
and gains
• The tumor suppressor genes—p53 and RB1.
Q 6. What is the order of investigation in this case?
Imaging is the first investigation of choice which
may be either CT or MRI for extremity and CT
358
Clinical Surgery Pearls
The biopsy should establish the grade and the
histologic subtype.
Q 9. What are the soft tissue sarcomas where
lymph node metastases are seen? (PG)
Soft tissue sarcomas with nodal metastasis
(< 3% of adult STS)
1. Synovial sarcoma
2. Ewing’s sarcoma
3. Embryonal rhabdomyosarcoma
4. Epithelioid sarcoma
5. Lymphangiosarcoma
6. Angiosarcoma
7. Kaposi sarcoma
8. Malignant fibrous histiocytoma (MFH).
Nodes are markers of systemic disease and need
radical node dissection.
Q 10. What are the common sites of soft tissue
sarcoma and what is the incidence?
It forms 1% of adult human malignancy and 15%
of pediatric malignancy.
Extremities – 50%(35%inlowerlimband15%
in upper limb)
Trunk – 31%
Head and Neck – 9%
Others –
Q 11. What are the most common histopathological subtypes?
1. MFH (the new terminology as per WHO is High
grade undifferentiated pleomorphic sarcoma
– 24%
2. Liposarcoma – 19%
3. Leiomyosarcoma – 21%
4. Synovial sarcoma – 12%
5. Nerve sheath tumors – 6%
6. Fibrosarcoma – 11%
7. Other types – 7%
Cell of origin Sarcoma type
Adipocyte Liposarcoma
Fibrohistiocyte Malignant fibrous histiocytoma
(High grade undifferentiated
pleomorphic sarcoma)
Fibroblast Fibrosarcoma
Smooth muscle Leiomyosarcoma
Skeletal muscle Rhabdomyosarcoma
Vascular Angiosarcoma, Kaposi’s
Synovial Synovial sarcoma
Unknown Ewing’ssarcoma,Epithelioidsarcoma
Q 12. What is the age group affected?
• Childhood – Embryonal
rhabdomyosarcoma
• Less than 35 years – Synovial sarcoma
• Older patients – MFH and liposarcoma
Q 13. Why the grade is important in STS?
Grade of the tumor is included in stage grouping.
Q14. What are the factors considered for grade?
They include:
• Cellularity
• Differentiation
• Pleomorphism
• Necrosis
• Number of mitosis.
Q 15. Based on mitotic activity how is grading done?
• Mitotic activity 0–9 / high power field
• 10 – 19
• 20 or more.
Q 16. What are the imaging studies of choice?
• Plain radiograph—underlyingskeletaldeformities,
callus and bony exostosis can be identified
• CT is preferred for intra-abdominal lesion
because one can identify both primary and
potential metastasis
Soft Tissue Sarcoma
359
• MRI—Best suited for accurate anatomical
localization
– Whetherlesionisintraorextracompartmental
– Can diagnose lipoma and hemangioma with
reasonable accuracy
– Identify the relationship of the sarcoma to
neurovascular structures.
• MR angiography (The role of arteriography has
decreased markedly after MR angiography)
• Ultrasound—can guide biopsy.
Useful under certain circumstances
1. PET CT scan may be useful for targeting biopsy,
in prognostication, grading and determining
response to preoperative chemotherapy. MR
spectroscopy is coming up in a big way for
grading of the tumor.
2. Consider abdominal/pelvic CT for the following
types of extremity soft tissue sarcoma:
– Myxoid liposarcoma
– Epithelioid sarcoma
– Angiosarcoma
– Leiomyosarcoma
3. MRI of spine for myxoid round cell liposarcoma
4. CNS imaging for alveolar sarcoma and
angiosarcoma.
Q 17. What is the timing of imaging?
Imaging is done prior to core biopsy and incision
biopsy. The core biopsy will produce architectural
alterations in the lesion.
Q 18. What is the metastatic work up?
• X-ray chest – 70% of the extremity sarcomas
metastasize to the lungs
– Retroperitonealorviscerallesionsmetastasize
to the liver parenchyma
• CT scan of the chest is recommended for high
grade tumors and for all tumors > 5 cm size.
Metastases are seen in the periphery of the
lung. It is superior to chest X-ray for identifying
metastasis for low grade tumors.
Q 19. What is the staging of soft tissue sarcoma?
(PG)
TNM Staging: AJCC 7th Edition.
• Grading has been reformatted from a four
grade to a 3 grade system as per the criteria
recommended by the College of American
Pathologists
• N1 disease has been reclassified as stage III
rather than stage IV.
Primary Tumor (T)
TX – Primary tumor cannot be assessed
T0 – No evidence of primary tumor
T1 – Tumor 5 cm or less in greatest dimension
T1a – Superficial tumor
T1b – Deep tumor
T2 – Tumormore than 5 cm in greatest dimension
T2a – Superficial tumor
T2b – Deep tumor
Regional Lymph Nodes (N)
NX – Regional lymph nodes cannot be assessed
N0 – No regional lymph node metastasis
N1* – Regional lymph node metastasis
*Note: Presence of positive nodes (N1
) is considered
Stage IV (the outcome of patients with N1 disease
is similar to those with M1 disease).
Distant Metastasis (M)
MX – Distant metastasis cannot be assessed
M0 – No distant metastasis
M1 – Distant metastasis
Histologic Grade
GX – Grade cannot be assessed
G1 – Grade 1
G2 – Grade 2
G3 – Grade 3
360
Clinical Surgery Pearls
Stage Grouping
Stage T size N size M Grade
IA T1a
T1b
N0
N0
M0
M0
G1, Gx
G1,Gx
IB T2a
T2b
N0
N0
M0
M0
G1, Gx
G1,Gx
IIA T1a
T1b
N0
N0
M0
M0
G2, G3
G2,G3
IIB T2a
T2b
N0
N0
M0
M0
G2
G2
III T2a, T2b
Any T
N0
N1
M0
M0
G3
Any G
IV Any T Any N M1 Any G
Q 20. What are the immunohistochemical markers
for soft tissue sarcoma? (PG)
IHC marker Type of sarcoma
Desmin Sarcoma from smooth,
skeletal muscle
CD 31, CD 34 Vascular sarcomas
S 100
Sarcoma with neural,
lipomatous, chondroid
differentiation
Vimentin Many sarcomas—non
specific
CD 117 Gastrointestinalstromial
tumor
CD 57, Vimentin Chondrosarcoma
CD 99, S 100, NET. Vimentin PNET/Ewing’s sarcoma
Cytokeratin, CD 99, NET
Desmin
Desmoplastic round cell
tumors
CD 57, EMA, Vimentin Osteosarcoma
Q 21. What is the staging proposed by Memorial
Sloan Kettering Cancer Centre (MSKCC)? (PG)
This is a simple staging system based on the three
important prognostic factors namely:
1. High grade – 1 point
2. Tumor size > 5 cm – 1 point
3. Deep tumor – 1 point
4.
Good prognostic
factor
Poor
prognostic
factor
Staging
Low grade High grade Stage 0 - no poor
prognostic feature
Stage 1 – 1 poor
prognostic feature
Stage 2 – 2 poor
prognostic feature
Stage 3– 3 poor
prognostic feature
Stage4–metastatic
sarcoma
Tumor size < 5cm Tumor size >
5cm
Superficial tumor Deep tumor
Q 22. What you mean by superficial and deep in
staging? (PG)
Superficial lesions—lesions not involving the
superficial fascia.
Deep:
a. Lesions deep to, or involves the superficial fascia
b. All intraperitoneal visceral lesions
c. Retroperitoneal lesions
d. Mediastinal (Intrathoracic lesions)
e. Pelvic sarcomas
f. Head and neck tumors.
Q 23. What is the metastatic potential of low grade
and high grade STS? (PG)
• Less than 15% risk of metastasis for low grade
tumors
• More than 50% risk for high grade tumors
Soft Tissue Sarcoma
361
Note: For retroperitoneal sarcoma liver is the
principalsite of metastasis. For extremity sarcoma,
lung is the principal site of metastasis.
Q 24. What are the sarcomas excluded from this
staging system? (PG)
• Kaposi’s sarcoma
• Dermatofibrosarcoma protuberans
• Infantile fibrosarcoma
• Angiosarcoma
• Sarcomas arising from the dura materincluding
brain
• Sarcoma arising in the parenchymatous organs
• Hollow viscera
• Inflammatory myofibroblastic tumor
• Fibromatosis (Desmoid tumor)
• Mesothelioma.
Q 25. What is fibromatosis?
Original fibrosarcoma grade I is now mentioned
as fibromatosis (Desmoid) and they are not in soft
tissue sarcomas.
Q 26. What is the staging in this case?
T2b, N0, M0 (Stage 3).
Q 27. What is the management of soft tissue
sarcoma?
Multidisciplinary approach is recommended
comprising expertise in the following specialties:
• Radiology
• Clinical oncology
• Surgical oncology.
Note: Limbsparing surgery ispreferred.Amputation
is performed in < 5–10% of cases.
Q 28. What is the surgical management of this
tumor?
Limb sparing, function preserving, margin free
(microscopically negative) wide excision is the
treatment of choice.
Q 29. What is wide excision?
It is a wide en-bloc resection to obtain 1 cm
uninvolved tissue in all directions for low grade
tumors and 2 cm in all directions for high grade
tumors (3-dimensional).A3-dimensional clearance
without seeing the tumor is achieved.
Note: All earlier scars, fine needle aspiration tracts
and biopsy areaswith hematoma shouldbe excised
en-bloc with the underlying tumor.
Q 30. What is “Pseudocapsule” in soft tissue
sarcoma? (PG)
The sarcomas grow in an expansive fashion, flattening
the normal soft tissue structures around them in a
concentric manner and creating a compression
zone of condensed and atrophic tissue. Outside
this zone lies edematous neovascularized tissue
called reactive zone. Together the compression and
reactive zones comprise the pseudocapsule.
Small tentacles (small finger-like extensions)
extend for variable distance from the parent lesion
perforatingthepseudocapsuletoformclinicallyoccult
depositsbeyondthepseudocapsule. Therefore, tumor
masses will be seen outside the pseudocapsule.
Q 31. What are the barriers for the infiltrative
growth of the sarcomas? (PG)
The expansive growth stops at the following
boundaries:
1. Fascial boundaries
2. Periosteal structures
3. Adventitia of the vessels
4. Nerve sheaths.
Note: Grossinvolvement of these structuresisseen
early (may become infiltrated).
Q 32. What is intralesional excision? (PG)
When you leave behind the pseudocapsule and
remove the lesion, it is called intralesional excision.
It is not recommended.
362
Clinical Surgery Pearls
Q 33. What is marginal excision? (PG)
Removal of tumor along with its pseudocapsule is
called marginal excision (Not recommended).
Q 34. What is compartment excision? (PG)
Compartment excisions are done for soft tissue
sarcomas of the extremities. Removal of muscle
bundles from origin to insertion and all other structures
in the compartment is called compartment excision.
This is also given up in favor of wide excision.
Q 35. What is the role of amputation in soft tissue
sarcoma?
There is a paradigm shift from radical amputation
to limb salvage procedure in the treatment of soft
tissue sarcoma.
• Amputation as an initial treatment did not
decrease the probability of regional metastasis
and did not improve the disease specific survival
and therefore, a limb sparing attitude is taken
• Patient preference when grosstotalresection of
the tumor is expected to render the limb nonfunctional
• Sarcomas involving bone or joint.
Note: Amputation should be performed one joint
above the tumor.
Q 36. When complete encirclement of major
neurovascular bundle occurs what should be the
surgical approach? (PG)
1. Nerve resection: It may be necessary to sacrifice
these structures and give braces for footdrop
after sciatic nerve resection, knee brace for
joint stability after loss of quadriceps function
secondary to femoral nerve resection.
2. Resection of a major artery followed by
saphenous vein graft or prosthetic graft for
restoration of arterial flow.
3. If 1 and 2 are not feasible do amputations.
Q 37. In the given patient what will be the
treatment option?
In the given case the wide excision will involve
removal of part of the quadriceps muscle, resection
offemoral nerve followed by knee brace forstability
of the knee. The femoral vessels are unlikely to be
encircled by the tumor.
Q 38. What is the role of adjuvant radiation
therapy?
Radiotherapy is not a substitute for suboptimal
surgery. There are three types of radiotherapy:
• Brachytherapy
• IORT
• XRT
The indications for radiotherapy are:
• High grade lesions
• Low grade lesions > 5 cm
• Margin positive
• Close soft tissue margin < 1 cm
• Recurrent sarcomas.
Q 39. What are the advantages and disadvantages
o f p re o p e ra t i ve ra d i o t h e ra py a n d t h e
indications? (PG)
Preoperative radiotherapy is indicated for stage II
and III disease. The advantages are:
• Reduces seeding in surgical manipulation
• Pseudocapsule may thicken and become
acellular, easing resection
• To tackle occult micrometastasis
• To do less radical surgery later on
• In patients with unresectable tumors for limb
sparing surgery later on
The disadvantages are:
• Wound healing problems—may need the help
of plastic surgeon
• Resection is possible only after 3–6 weeks
The dose of radiotherapy is 50 Gy.
Soft Tissue Sarcoma
363
Q 40. What is the role of brachytherapy? (PG)
Adjuvant brachytherapy is being used increasingly
nowadays. Brachytherapy willtreatthe tumor bed,
within 2 cm of the margin. Radioactive wires are
placed into the operative bed to improve local
control. It will not treat large margins, overlying
skin, and scar or drain site. It has got a short duration
of treatment (4–6 days) compared to the external
beam therapy consisting of 6–8 weeks duration.
Q 41. What are the indications for chemotherapy? (PG)
• High grade liposarcoma
• High grade synovial sarcoma
• Ewing’s sarcoma
• Rhabdomyosarcoma.
Q 42. What is rationale for preoperative chemotherapy? (PG)
• Preoperative chemotherapy is indicated for
stage II and III
• To limit the spread of tumor at the time of
surgery.
Q 43. What are the chemotherapeutic regimens?
• The single agents used are:
– Doxorubicin, Ifosfamide and Dacarbazine
• Combination
– Gemcitabine and Docetaxel combination
– MAID: Mesna, Adriamycin, Ifosfamide,
Dacarbazine
Q 44. What is the treatment protocol for the
various stages?
Itis managed by multidisciplinary team—Surgeon,
Radiation Oncologist and Medical Oncologist. The
diagnosis is established by a carefully planned
biopsy which is done after imaging. Establish the
grade of the tumor and histological type before
treatment. Limb sparing, function preserving,
margin free wide excision is the surgical procedure
of choice.
Treatment protocol based on the staging:
Stage 1A (T1a - 1b N0, M0) /Stage IB:
• Wide excision—final margin > 1cm or intact
fascial plane—follow-up.
• Final margin < 1cm or without intact fascial
plane—consider radiotherapy
• Follow-up evaluation for rehabilitation, chest
imaging every 6–12 months
• Stage II and III Resectable/potentially resectable
disease
• Surgery/preoperativeradiotherapy/preoperative
chemotherapy, preoperative chemoradiation
followed by surgery
• RT/ considerRTboost/ consider adjuvant chemotherapy
• Follow-up.
Unresectable primary disease:
• RT/Chemotherapy/Chemoradiation/isolated
regional limb therapy
• Changes to resectable—surgery
• Unresectable—definitive RT/Chemotherapy/
Palliative surgery
• Follow-up.
Stage IV–Metastasis:
• Singleorgan/limitedtumorbulk –primary tumor
management and metastatectomy and RT/
chemotherapy and follow-up
• Disseminated metastasis—palliative chemo/
palliative surgery/palliative RT.
33 Neurofibroma, von
Recklinghausen's Disease
Case
Case Capsule
A 14-year-old mentally retarded boy presents with
2 subcutaneous swellings of anterior chest wall,
multiple café-au-lait macules (CALMs) and thoracic
scoliosis. There is a soft subcutaneous nontender
hanging swelling in the left mammary region of
20 × 15 cm size, freely mobile over the pectoralis
major muscle. The second swelling is of the size of 3 ×
4 cm in the region of the right 5th rib in the anterior
axillary line. The plane of this swelling is also subcutaneous. There is no axillary, cervical or inguinal
lymph adenopathy. There is a bony deformity
involving the 3rd, 4th and 5th ribs on right side.
Ophthalmologic examination showed Lisch nodules.
Radiology revealed hypoplastic sphenoid wings,
posterior scalloping of vertebral bodies, twisted
ribbon-like ribs and mediastinal swelling.
Read the diagnostic algorithm for a swelling.
Checklist for history
• Family history of similar swelling, involvement of
1st degree relative with NF1
• History of rapid increase in size suggestive of
malignancy
• History of headache
• History of seizures
• History of learning disabilities
• History of precocious puberty
• Historyofpainradiatingdownthe swelling(tingling
and numbness).
Checklist for examination
Read checklist for examination of the swelling.
1. Look for café-au-lait macules (CALMs)—6 or more
in number each having > 1.5 cm size during post
pubertal period
2. Look for freckling of axilla and groin
3. Look for more swellings
4. Rule out short stature
5. Examination of the spine for scoliosis and other
vertebral anomalies
6. Examination of the long bones for bony
abnormalities-pseudoarthrosis, etc.
7. Examination of the abdomen for pheochromocytoma
8. Record the blood pressure to rule out pheo
9. Complete neurological examination—both central
nervous system and peripheral nervous system
(including cognitive impairment, headache, etc.)
10. Ophthalmological examination to rule out Lisch
nodules in Iris—2 or more Lisch nodules
11. Rule out hydrocephalus. Contd...
Contd...
Neurofibroma, von Recklinghausen's Disease
a mixture of neural (ectodermal) and fibrous 365
(mesodermal) elements.
Q 3. What is the histology of neurofibroma?
There is a generalized neoplastic activity within the
nerve sheath. Histologically connective tissue and
endoneural cells intermixed with axons are seen.
Q 1. What is the most-probable diagnosis in this
case?
von Recklinghausen’s disease (neurofibromatosis)
Q 2. What is neurofibroma?
Neurofibromas are benign tumors which contain
366
Clinical Surgery Pearls
Q 7. What are the investigations for neurofibroma?
Investigations for neurofibroma
1. MRI—showing Gadolinium enhancing mass
within a peripheral nerve
2. Nerve conduction studies
3. Electromyography.
Q 8. What is the treatment of neurofibroma?
• Neurofibromas cannot be excised without
resecting a segment of the involved nerve.
• True neurofibromas should only be biopsied
to ensure that they are not malignant and
should be left in place unless the parent nerve
can be sacrificed without producing significant
neurological deficits.
Q 9. What is neurilemmoma? What is schwannoma?
• Tumorswhich are derived from the sheath ofthe
nerve are called neurilemmoma.
• Schwannoma—When the tumor is arising
from the Schwan cells it is called schwannoma.
They are benign, painless firm nodules of
1–2 cm size seen along the peripheral nerves.
They are usually asymptomatic with no nodal
involvement or malignant potential.
• They can be carefully enucleated by incising the
nerve sheath vertically without sacrificing the
nerve.
Q 10. What are the clinical features of neurilemmoma and schwannoma?
They are having the same clinical features as
neurofibroma.
Q 11. What is the treatment of schwannoma?
• Generally they are small and benign and
they can be enucleated by incising the nerve
sheath.
• Amputation may be required for malignant
schwannoma.
Q 4. What are the diagnostic clinical features of
neurofibroma?
Diagnostic features of neurofibroma
• They are seen in relation to a nerve (Need not be in
relation to named nerve)
• Patients may get tingling sensation in the
distribution of the nerve
• They are usually seen in the subcutaneous tissue
and skin (Deep swelling can occur)
• Usually fusiform in shape
• Long axis of the swelling is along the length of the
limb
• Firm or rubbery in consistency
• Mobile side-to-side (not longitudinally)
• Moves at right angles to the course of the nerve
• No nodal involvement
• Skin is not involved
• Nerve compression symptoms may be there.
Note: Deep neurofibromas in the extremities
and intra-abdominal neurofibromas may not be
showing the classical features.
Q 5. When neurofibromas are multiple what is it
called?
• Neurofibromatosis
• When they are multiple and congenital and
familial and transmitted by autosomal dominant
gene, it is called von Recklinghausen’s disease.
Q 6. What are the manifestations of neurofibroma?
It can be classified as:
• Irritative—pain distributed along the course of the
nerve
• Paralytic
• Sensory dysfunction
• Motor dysfunction—weakness and muscle atrophy.
Neurofibroma, von Recklinghausen's Disease
367
Q 12. What are the types of neurofibromas?
Three types of neurofibromas are there:
1. Peripheral neurofibromas
2. Diffuse or plexiform neurofibromas – 5% undergo
malignant transformation resulting in MPNST.
3. Malignant peripheral nerve sheath tumors (MPNSTs).
Q 13. What are the types of plexiform neurofibromas?
There are 3 types of neurofibromatosis:
1. Plexiform neurofibromatosis—It is an excessive
over growth of neural tissue in the subcutaneous
fat and makes the tissue look edematous. They
are usually seen in connection with the branches
of 5th cranial nerve. This will present as mass of
soft tissue hanging down in folds.
2. Elephantiasis neuromatosis—It is a variant of
the above condition affecting the lower limb
where the subcutaneous fat is replaced by
fibrous tissue. It is often mistakenly diagnosed
as lymphedema, but the lymphatics are normal.
3. Pachydermatocele— I t i s a t y p e o f
neurofibromatosis in which coils of soft tissue
hang around the root of the neck.
Q 14. What is amputation neuroma?
A tumor similar to neurofibroma occurring at the
end of the divided nerve in amputation is called
amputation neuroma. Such neuromas may also
be seen where a divided nerve has failed to unite.
Q 15. What are the types of neurofibromatosis?
They are classified as:
A. Isolated neurofibromas
B. Multiple neurofibromas (Neurofibromatosis)—
further classified as—
1. Neurofibromatosis—Type I (NF - I) (von
Recklinghausen’s disease),
2. Central neurofibromatosis—NF Type II.
Q 16. What is central neurofibromatosis (type II)?
• Itis characterized by bilateral acoustic neuromas
and spinal neuromas
• Always check the hearing
• Always check nerve root compression when you
suspect spinal neuromas
• This condition also shows autosomal dominant
inheritance.
Q 17. What is dumbbell tumor?
Spinal tumors may be dumbbell shaped and part of
it may be inside and part of it outside the vertebral
canal. It may cause nerve root compression.
Q 18. What is café-au-lait spot?
They are patches of pale brown pigmentation. The
diagnostic criterion for café-au-lait spot is that they
should be greater than 6 in number and more
than 1.5 cm size across. They are characteristic
and diagnostic of von Recklinghausen’s disease.
Q 19. What are the diagnostic criteria for von
Recklinghausen’s disease (NF-1)?
The NIH consensus criteria are used. The diagnosis
ofNF-1 isrenderedwhen 2 or more of the following
are present.
Diagnostic criteria for von Recklinghausen’s
disease (2 or more must be there)
1. Six or more café-au-lait spots. Each 1.5 cm or larger
in postpubertal individuals, 0.5 cm or larger in
prepubertal individuals
2. Two or more neurofibromas of any type OR one or
more plexiform neurofibromas
3. Freckling of armpits or groin
4. Optic glioma (tumor of the optic pathway)
5. Two or more Lisch nodules (benign iris hamartomas)
6. A distinctive bony lesion—Dysplasia of the
sphenoid bone/dysplasia or thinning of long bone
cortex
7. First degree relative with NF-1.
368
Clinical Surgery Pearls
Q 20. What are the pigmentary abnormalities in
NF-1?
a. Café-au-lait macules (CALMs)
b. Skin fold freckling
– < 5 mm in size, not apparent at birth
– appears later in childhood.
c. Lisch nodules—(pathognomonic).
Q 21. What is skin fold freckling?
T h e s e c o n d m o s t c o m m o n p i g m e n t a r y
abnormality NF-1 is skin fold freckling seen in
the axilla, groin and intertriginous non sun
exposed areas. It is also seen under the chin
and inframammary regions in adults. Unlike
café-au-lait spot these are < 5 mm in size and not
apparent at birth.
Q 22. What is Lisch nodule?
It is pathognomonic for NF-1. Lisch nodules are
raised, pigmented hamartomas of the iris. They do
not interfere with vision and are not associated with
any clinical symptoms.
Q 23. What are the malignancies associated with
NF-1?
1. Optic gliomas (the most commonly recognized
tumor)
2. Astrocytomas
3. Pheochromocytoma—Check BP, do ultrasound
abdomen and urinary VMA for excluding pheo
in all cases of NF1.
4. Non CNS malignancies—Leukemias (juvenile
chronic myeloid leukemia, and myelodysplastic
syndromes)
Among individuals with NF-1 pheo is rare,
however in patients with pheo the incidence of
NF-1 is estimated to be between 4–23%.
Q 24. What are the other neurological complications? (PG)
Other neurological complications of von
Recklinghausen’s disease
• Macrocephaly
• Hydrocephalus—Aqueductal stenosis
• Cognitive impairment
• Headaches
• Seizures (5–7%)
• Cerebral ischemia
• Learning disabilities
• Mental retardation (IQ < 70)
• Diffuse cerebral dysgenesis—10%
• Cerebrovascular abnormalities producing strokes.
Q 25. What are the bony abnormalities? (PG)
Bony abnormalities in von Recklinghausen’s disease
• Dysplasia of sphenoid
• Scoliosis—10% of the affected individuals
• Short stature
• Vertebral defects—hemivertebrae
• Long bone deformities Tibial dysplasia (anterolateral
bowing)—thinning of the cortex leading to
pathological fracture and non union—pseudoarthrosis
• Twisted ribbon-like ribs.
Q 26. What is the genetics of von Recklinghausen’s
disease? (PG)
• Itis adisease having autosomal dominantinheritance pattern.
• 30–50% do not have an affected parent
(spontaneous mutation)
• The penetrance of NF1 is essentially 100% in
individuals who have reached adulthood.
• The protein product of the NF1 gene is called
Neurofibromin
• The gene is located in chromosome 17q 11.2.
(The NF2 gene is called Merlin).
Neurofibroma, von Recklinghausen's Disease
369
Q 27. What is the management of von Recklinghausen’s disease? (PG)
The NF1 management is teamwork of physicians,
ophthalmologists, neurologists, orthopedic surgeons,
dermatologists, oncologists, otolaryngologists, plastic
surgeons, neurosurgeons, psychiatrists, social workers
and child psychologists. This is better accomplished
by NF clinic.
Optic pathway gliomas
• Serial neurologic and ophthalmologic examination
• MRI scan once tumor is identified (ophthalmic
examination should be performed on all
children starting at 1 year and continuing
annually for at least the first decade).
Plexiform neurofibromas
• Needs regular follow-up because of the
malignant transformation.
Scoliosis
• Requiresorthopedic evaluation/bracing/surgery
Tibial dysplasia
• Orthopedic management
Learning disabilities
• Early intervention
Precocious puberty
• Requires management by endocrinologists.
Q 28. What is the role of genetic counseling? (PG)
Issues about genetic transmission, emotional aspect
and what can be expected to come in future are
discussed with the family.
Q 29. What is likely to be the future management?
(PG)
Development of targeted treatment is likely to be
the future treatment. The application of drugs that
interfere with RAS proto-oncogene activity would
be predicted to have beneficial effects.
Q 30. What surgical treatment you offer for the
given patient?
Thetwochestwallswellings(Plexiformneurofibromas)
will be excised under general anesthesia and the
patient will be kept for regular follow-up.
Diagnostic algorithm for a swelling anywhere
1. Identify the anatomical situation of the swelling (In
relation to the triangle in the neck)
↓
2. Decide the plane of the swelling
↓
3. Recollect your anatomy (What are the normal
anatomical structures situated in the region of the
swelling in that plane?)
↓
4. Check for mobility/fixity of the swelling
↓
5. Find out the external (size, shape, surface, edge,
temperature, tenderness, etc.) and internal features
of the lump (solid or cystic, compressible/ reducible,
pulsation, transillumination) and auscultation of
the swelling.
↓
6. Find out its effect on the surrounding tissue
↓
7. Come to an anatomical diagnosis
↓
8. Come to a pathological diagnosis (Decide whether it
is congenital/traumatic/ inflammatory / neoplastic)
↓
9. If it is an organ concerned with function decide
whether it is hyperfunctioning, normally
functioning or hypofunctioning (functional
diagnosis). The final diagnosis = Anatomical +
Pathological + Functional diagnosis.
34 Lipoma (Universal Tumor)
Case
Case Capsule
A 40-year-old female patient presents with a
swelling of the size of 5 × 4 cm on the right side of
the back of 3 years duration. On examination, the
swelling is soft and lobulated with a slipping edge
situated in the infrascapular region. The swelling
is mobile. There are no signs of inflammation or
malignancy. No axillary node involvement is noted.
No other swellings detected clinically.
Read the diagnostic algorithm for a swelling.
Q 1. What is your clinical diagnosis in this case?
Lipoma.
Q 2. What is lipoma?
It is a benign tumor arising from adult fat cells.
lipoma back
Q 3. What are the diagnostic points for lipoma?
1. Lobulation
2. Slip sign
3. Soft swelling with pseudofluctuation
4. Transillumination positive if it is subcutaneous
5. The overlying skin may show prominent veins
when the lesions are large.
Q 4. What is the cause for pseudofluctuation?
Intracellular fat is fluid at body temperature.
Therefore, the swelling will be soft and fluctuation
will be elicited in one plane. For a true cyst
one should elicit fluctuation in two planes at
right angles to each other that is not possible
in the case of lipoma and therefore, it is called
pseudofluctuation.
Q 5. What is slip sign?
If the edge of the lump is pressed, the swelling
slips from beneath the finger. This can be easily
demonstrated in the case of a subcutaneous lipoma
and it is said to be pathognomonic.
Lipoma (Universal Tumor)
371
demonstrated in subcutaneous lipoma. It is not
possible to demonstrate, slip sign and lobulation
in deep lipomas.
Q 12. What will be the finding in transillumination test?
In subcutaneous lipomas transillumination may
be positive. Big lipomas and deep lipomas are not
transilluminant.
Q 13. What are the diagnostic points for intermuscular lipoma?
On contraction of the concerned muscle the lipoma
becomes more prominent, if it is intermuscular. If
it is deep to the muscle, the lipoma will become
less prominent.
Q 14. What is Nevolipoma?
When a lipoma contains dilated capillaries, it is
called Nevolipoma. These swellings will be partially
compressible.
Q 15. What is Dercum’s disease (Adiposis
dolorosa)?
It is a condition where multiple painful or tender
subcutaneous lipomas are seen. It is called adiposis
dolorosa. It is also called neurolipomatosis.
Q 16. What is fibrolipoma?
When lipoma contains much fibrous tissue it is
called fibrolipoma.
Q 17. What are the symptoms of lipoma?
1. Swelling (lump)
2. Cosmetic (unsightly swelling)
3. Interfere with movement
4. Pain (due to trauma and fat necrosis).
Q18. What are the complications of lipoma?
1. Myxomatous degeneration
2. Calcification
3. Pressure effects
Q 6. How will you demonstrate lobulation?
The swelling is compressed between the finger
and thumb of one hand, while its surface that is
now made more prominent, is stroked firmly by the
fingers of the other hand.
Q 7. What is the cause for lobulation?
The lobulation is because of the intervening fine
strands of fibrous septa seen between the fat lobules
(collection of overgrown fat cells). The lobules bulge out
between the fibrous strands when pressure is applied.
Q 8. Why it is called universal tumor or ubiquitous
tumor?
Lipomas can occur anywhere in the body where fat is
present and therefore, it is called universal tumor. The
aphorism “When in doubt hedge on fat” is still true.
Whenever you do not have a diagnosis for a given
swelling, lipoma is one of the differential diagnoses.
Q 9. Depending on the plane, how will you classify
lipomas?
Lipomas can be classified as:
• Subcutaneous • Intra-articular
• Subfascial • Subserous
• Intermuscular • Subperitoneal
• Intramuscular • Subpleural
• Paraosteal • Subpericardial
• Subperiosteal • Extradural (type of spinal
tumor)
• Subsynovial • Submucous
• Intraglandular—seen in breast, pancreas, under
renal capsule, etc.
Q 10. What is the commonest plane of lipoma?
Subcutaneous (between the deep fascia and skin).
Q 11. Is it possible to demonstrate all the classical
signs of lipoma when the swelling is deep?
No. The classical signs of lipoma such as slip
sign, lobulation and pseudofluctuation, etc. are
372
Clinical Surgery Pearls
Q 20. What are the investigations required?
1. FNAC from the swelling
2. Radiology of the part
3. If the swelling is big and sarcoma is suspected,
core biopsy and MRI are done.
Q 21. What is the surgical treatment recommended in the given case?
Excision of the swelling under general anesthesia
(if the swelling is small excision is done under local
anesthesia using 1% lignocaine with or without
adrenaline).
4. Fat necrosis (if they are situated over prominent
areas and subjected to trauma)
5. Ulceration (repetitive friction cause ulceration)
6. Intussusception in submucous lipoma.
Note: Lipomas never turn malignant. Liposarcomas
arise de novo and not in a benign lesion.
Q 19. Which are the areas where liposarcomas are
commonly seen?
1. Proximal thigh
2. Retroperitoneum
3. Mediastinum.
35 Sebaceous Cyst/Epidermoid Cyst/
Wen/Dermoid Cyst
Case
Case Capsule
A 45-year-old male patient presents with a swelling
of size of 3 × 2 cm on the occipital region of the scalp
of 4 years duration. On examination, the swelling is
soft and cystic. The sign of indentation is present.
A punctum is visible at the summit of the swelling.
The swelling is mobile. The skin can be pinched all
over the swelling except at the punctum. There are no
signs of inflammation or malignancy. No lymph node
involvement. No other swellings detected clinically.
Read the diagnostic algorithm for a swelling.
Checklist for examination
• Look for punctum
• Pinch the skin and decide whether the skin is free
or not (decide the plane)
• Look for fluctuation
• Try transillumination
• Look for the sign of indentation
• Decide whether it is cystic or solid
• Decide whether the swelling is pulsatile or not
• Lookforadepression in the bone or defect in the bone
• Look for cough impulse (to rule out intracranial
communication)
• Check the mobility (if itis bony it will not be mobile)
• Look for regional nodes.
374
Clinical Surgery Pearls
Q 9. What is the “sign of indentation”?
Cysts containing pultaceous material can be
moulded. When the swelling is indented with a
finger, it stays indented in contradistinction to the
sign of emptying.
Note: Pultaceous (Latin) = porridge.
Q 10. What are the conditions producing sign of
indentation?
Conditions producing sign of indentation
• Lax sebaceous cyst
• Large dermoid
• Solid feces in sigmoid in the left iliac fossa.
Q 11. What is punctum?
The cyst is arising basically from a skin structure
(sebaceous gland). The mouth of the sebaceous
gland will open into the hair follicle or through a
fine duct directly into the skin surface. This point of
fixation is pulled inwards as the cyst grows to form
the punctum. Gentle squeezing of the skin over the
cyst will demonstrate the punctum.
Q 12. What are the classical sites for sebaceous
cyst?
• It canoccurwhereverthereare sebaceousglands
• They are found in the hairy parts of the body.
The sites are:
Classical sites for sebaceous cyst
• Scalp
• Scrotum
• Back
• Shoulders
• Neck
• Face.
Q 1. What is your clinical diagnosis in this case?
Sebaceous cyst.
Q 2. If the punctum is not there, what are the
differential diagnoses?
1. Dermoid cysts.
2. Pulsatile bony swelling—primary/secondary.
Q 3. What are the primary pulsatile bony swellings?
1. Solitary plasmacytoma
2. Telangiectatic variety of osteogenic sarcoma.
Q 4. What are the causes for pulsatile bony
metastasis in this region?
1. Metastasis from follicular thyroid cancer
2. Renal cell carcinoma metastasis.
Q 5. What is sebaceous cyst?
The skin is normally kept soft and oily by the
sebum secreted by the sebaceous glands, mouth
of which open into the hair follicles. If the mouth
of a sebaceous gland is blocked, the gland will
get distended by its own secretions producing
sebaceous cyst.
Q 6. What is the plane of sebaceous cyst?
• All sebaceous cysts are attached to the skin
• The area of attachment may be small
• There is no independent movement of the cyst
of the skin
• Partofthe cystwill lie inthe subcutaneoustissue.
Q 7. What is wen? Is there any other synonym
for wen?
• Itis a synonym forthe sebaceous cyst ofthe scalp
• The other synonym is epidermoid cyst.
Q 8. Do the punctum exist in all cases?
Only 50% will have visible punctum. However all
sebaceous cysts are attached to the skin. Punctum
is some time difficult to demonstrate especially
in the scalp.
Sebaceous Cyst/Epidermoid Cyst/Wen/Dermoid Cyst
375
Q 19. What is Cock’s peculiar tumor?
Suppurating and ulcerating sebaceous cyst
is called Cock’s peculiar tumor. It looks like a
squamous cell carcinoma (SCC).This eponym isstill
used by surgeons for sentimental reasons.
It is an open, granulating and edematous
sebaceous cyst. The granulation tissue arises from
the lining of the cyst giving the lesion an everted
edge. Because of the infection, the whole area is
edematous, red and tender. The regional nodes may
be enlarged unlike sebaceous cyst.
Q 20. What will be the history in such a case?
The usual history will be a long duration of swelling,
followed by pain and discharge of pus from the
swelling or history of inadequate incision of the
swelling.
Q 21. How will you manage a sebaceous cyst?
1. FNAC
2. X-ray especially, if it is situated in the scalp (to rule
out bony defect that will be seen in dermoid cyst
or to rule out bone destruction seen in metastasis)
3. Rule out diabetes mellitus by doing blood sugar
estimation.
Q 22. What is the surgical treatment of sebaceous
cyst?
Excision under local anesthesia, using 1% or 2%
lignocaine.
Q 23. What is the incision recommended?
An elliptical incision. The ellipse will encircle the
punctum, so that the area of skin bearing the
punctum is removed, along with the cyst. Try to
remove the cyst intact along with the entire cyst wall.
Q 24. What is the treatment of infected sebaceous
cyst?
• If frank pus is formed, incision and drainage is
recommended, like any other abscess
Q 13. What is the age group affected?
• Rare before adolescence
• May appear suddenly during adolescence
• Most of them are seen in early adulthood and
middle age.
Q 14. Mention two sites where sebaceous cyst
will not occur?
• Palms
• Soles.
Note: There are no sebaceous glands in these regions.
Q 15. What is the syndrome associated with
sebaceous cyst?
Gardner’s syndrome
• Osteomas
• Intestinal polyposis
• Sebaceous cyst.
Q 16. What is the content of the cyst?
Toothpaste like, whitish, granular material with
unpleasant smell (pultaceous).
Q 17. What are the complications of sebaceous
cyst?
Complications of sebaceous cysts
• Infection
• Sebaceous horn
• Cock’s peculiar tumor.
Q 18. What is sebaceous horn?
The slow discharge of sebum from a wide punctum
hardens to form the horn. This will take the shape
of a conical spike. Normally horn is not formed
because soap and water and friction from cloths
will remove the secretion of the gland. Failure to
wash the skin over the cyst will result in horn. Wide
punctum (opening) is seen after an infected cyst has
ruptured. The horn can be broken off.
376
Clinical Surgery Pearls
• If it is inflammation alone, give a course of
antibiotics and do elective excision.
Q 25. What will happen to the cyst after incision
and drainage?
It will recur and need formal excision later on.
Q 26. What will be the histopathological feature
of sebaceous cyst?
The content will be toothpaste like and fowl smelling
and it will be lined by squamous epithelium.
Q 27. What are the differences between sebaceous
cyst and dermoid cyst?
Sl. No. Features Sebaceous cyst/Epidermoid cyst/Wen Dermoid cyst
1. Cause Due toblockage of duct ofsebaceous gland Congenital—sequestration of epithelial
elements deep to the skin surface
2. Site Hair bearing site on the body—back, face,
neck, scalp, scrotum
Along the lines of embryological skin
fusion. External and internal angular
dermoids, sublingual (superficial/deep),
postauricular, pre and postsacral
3. Skin-involvement Present (tethering) Not involved
4. Punctum Present Absent
5. Lining Sebaceous cells/squamous epithelium Skin with appendage, i.e. sebaceous
glands, hair follicles and hair-(keratinized,
stratified squamous epithelium)
6. Content Toothpaste like, whitish, fowl smelling
material
Hair and sebaceous material
7. Intracranial
communication
No communication Communication may be present
8. Bony defect No bony defect Bony defect may be seen
9. Palms and soles No hair follicles and do not occur Occur, e.g. implantation dermoid
Q 28. What are the classical sites of dermoid cysts?
• Dermoid cysts may be congenital or acquired.
• The example of acquired dermoid cyst is
implantation dermoid, seen in the hands and
feet as a result of trauma
• Congenital dermoid cysts are seen along the
lines of embryological fusion:
Sites where congenital dermoid cysts are seen
• Midline of the body
• Sites where two embryonic processes meet:
a. Outer angle of the orbit (frontonasal process and
maxillary process fuse in this region)—External
angular dermoid
b. Behind pinna (Postauricular)
c. Below the tongue (sublingual dermoid)
d. Pre and postsacral dermoid.
Sebaceous Cyst/Epidermoid Cyst/Wen/Dermoid Cyst
377
Q 29. Why is the dermoid cyst at the outer angle of
the orbit called external angular dermoid?
That is because it lies behind the outer end of the
eyebrow, over the external angular protuberance
of the skull. This is a congenital dermoid cyst.
Q 30. Can dermoid cyst occur at the inner end of
the eyebrow (medial end of the eyebrow)?
Yes. It is called internal angular dermoid.
Q 31. What are the complications of external
angular dermoid?
1. Bony depression—by pressure
2. Dumbbell extension into the orbit
3. Erosion of the orbital plate of frontal bone and
getting attached to the dura.
Q 32. What is the peculiarity of postsacral
dermoid?
It may expand within the spinal canal causing
compression of the cauda eqina. The lesion may be
present at birth but usually noted in the first two
years and occasionally may be seen in adulthood.
Q 33. What is the cause for implantation dermoid?
They are usually traumatic. The surface ectoderm is
being driven inside as a result of trauma (small cut
or stab injury) and this will result in implantation
dermoid. They are usually seen in fingers and toes.
They may be sometimes tense and hard. History of
old injury or presence of a scar will give the clue.
Q 34. What are the classical features of dermoid cyst?
Diagnostic features of dermoid cyst
1. Cystic swelling
2. Not transilluminant
3. The skin can be pinched (no punctum and therefore
no tethering)—They are seen deepto the skin in the
subcutaneous tissue
4. A bony depression or defect may be felt
5. Intracranial communication may be there.
36 Ulcer
Case
Case Capsule
A 20-year-old male athlete presents with an ulcer
of size of 6 × 3 cm over the shin of the leg of 6
months duration. He gives history of trauma to
the shin region while playing followed by the
development of ulcer. On examination, the floor
of the ulcer is covered with red granulation tissue
surrounded by a blue line outer to the granulation
and white line further outside. There is minimal
serous discharge. The ulcer is mobile and there
is no fixity to the deeper structures. The vertical
group of inguinal nodes are enlarged and tender.
On examination, there is no evidence of varicose
veins. The peripheral pulsations are normal. There
are no sensory deficits and no other neurological
problems. Systemic examination is normal.
Checklist for examination of ulcer
1. Examine the site, edge floor, base and
surrounding tissue (SEFBS)
2. Look for fixity to underlying structures (check the
movements of the ulcer)
3. Look for peripheral pulsations (arterial ulcer)
4. Look for varicose veins (venous ulcer)
5. Check for sensations (neuropathic ulcers)
6. Look for nerve thickening, hypopigmented
patches and other stigmata of Hansen’s disease
(to rule out Hansen’s disease)
7. Look for movements of the joints and deformities
(deformity of the foot in DM equinus deformity in
venous ulcer and deformity of Paget’s disease of
the bone)
8. Examine the regional nodes (vertical group of
inguinal nodes are involved in lower limb ulcers)
9. Look for stigmata of syphilis
10. Examine for lymph nodes of neck, axilla, and
inguinal region
11. Examination of the chest, to rule out tuberculosis
12. Rule out diabetes mellitus
13. Exclude anemia (including sickle cell anemia) and
leukemia
14. Look for features of rheumatoid arthritis
15. Exclude Paget’s disease of the bone
16. General neurological examination to rule out
nervous diseases, spinal injuries, sciatic nerve
injuries, etc. Contd...
Contd...
Ulcer
379
380
Clinical Surgery Pearls
Q 7. What are the characteristics of spreading ulcer?
Characteristics of spreading ulcer
• The surrounding skin of the ulcer is inflamed and
edematous
• The floor is covered with slough
• No evidence of granulation tissue
• Discharge of pus will be there.
Q 8. What are the characteristics of callous ulcer?
Characteristics of callous ulcer
• Induration of the edge and surrounding tissue
• The floor will have pale granulation tissue
• No tendency towards healing
• Copious serous discharge.
Q 9. What are the causes for nonspecific ulcers?
The causes for nonspecific ulcers are:
1. Traumatic—(Footballers ulcer)
• Physical—Electrical
• Chemical—Caustics
• Mechanical—Dental ulcer, pressure from
splint, etc.
2. Arterial— Atherosclerosis, TAO, Raynaud’s disease
3. Venous—Venous ulcer, postthrombotic ulcer,
gravitational ulcer
4. Neuropathic—(Neurotrophic or Perforating)
Diabetes, leprosy, tabes dorsalis, spina bifida,
paraplegia, syringomyelia
5. Tropical ulcers—Tropical countries
6. Metabolic—Diabetes gout
7. Secondary to diseases like:
– Rheumatoid arthritis
– Erythrocyanosis frigida
– Osteitis deformans (Paget’s disease of the
bone)
– Avitaminosis
Q 1. What is your diagnosis?
A healing traumatic ulcer.
Q 2. Why healing ulcer? Why traumatic?
Healing ulcer because:
• The floor is covered with red granulation
• The blue line outside the granulation is
suggestive of growing epithelium
• The white line is suggestive of fibrous tissue
• There is no evidence of infection.
Traumatic because:
• Noevidenceof arterial, venous,neurological and
other systemic problems
• Young athlete
• History of trauma is present.
Q 3. What is ulcer?
The ulcer is a break in the continuity of an epithelial
surface that can occur in the skin or mucosa of the
alimentary or respiratory passages.
Q 4. What is slough?
Slough is a piece of dead tissue.
Q 5. What is the classification of ulcer?
Ulcer may be classified as:
1. Acute or chronic
2. Painful or painless (malignant ulcers are painless
in the early stages)
3. Spreading ulcer or healing ulcer or callous ulcer
4. Nonspecific ulcer or specific ulcer or malignant
ulcer.
Q 6. What are the characteristics of healing ulcer?
Characteristics of healing ulcer
• The surrounding skin is not inflamed
• Theedgeshowsblue zone (bluish outline of growing
epithelium) and a white zone (fibrosis of the scar)
• Floor will have reddish granulation tissue
• Minimal serous discharge may be there.
Ulcer
381
8. Ulcers complicating blood diseases:
– Sickle cell anemia
– Mediterranean anemia
– Felty’s syndrome (look for spleen)
9. Ulcer occurring on paralyzed leg—usually
seen in anterior poliomyelitis
10. Factitious ulcer (artefact ulcer)
11. Diphtheritic desert sore
12. Yaws
13. Decubitus ulcer
14. Iatrogenic ulcer—extravasation of IV fluid
15. Ulcers in congenital arteriovenous fistula
16. Miscellaneous—Martorell’s ulcer.
Q 10. What are the specific types of ulcers?
Examples of specific ulcers
• Tuberculosis
• Syphilitic
• Actinomycosis (bacterial)
• Soft sore
• Herpes simplex
• Fungal.
Q 11. What are the types of ulcers seen in syphilis?
• Hunterian chancre (hard chancre)—Seen in
primary syphilis
– About 3–4 weeks after exposure in the
external genitalia
– Ulcer is painless
– Lymph nodes are enlarged
• Gumma (lymph nodes are not involved)—Seen
in tertiary syphilis
– Seen in subcutaneous bones like tibia,
sternum, skull, and ulna
– In relation to the testis
– In the leg.
Q 12. What are the lesions in the secondary
syphilis?
• Mucouspatches—Soddenthickenedepithelium
• Condylomas—Raised flat, white hypertrophied
epithelium at the mucocutaneous junction
(angles of mouth, anus, and vulva)
• Enlarged lymph nodes—Epitrochlear, and suboccipital nodes.
Q 13. What is soft sore or soft chancre?
It is also called Ducrey’s ulcer. They are multiple
acute ulcers with yellowish slough seen in the
external genitalia appearing 3 days after infection.
They have copious purulent discharge. The regional
lymph nodes are enlarged.
Q 14. What are the types of edges for the ulcer?
Edges may be:
Various types of edges of ulcer with examples
Sl no Type of edge Examples
1. Sloping edge Healing ulcers, venous
ulcers
2. Punched out edge Syphilitic, trophic,
ischemic and leprosy
3. Undermined edge Tuberculosis, bedsore,
carbuncle
4. Raised edge Rodent ulcer (basal cell
carcinoma)
5. Everted edge Squamous cell carcinoma
(old name–epithelioma)
Q15. What is the cause for undermined edge in
tuberculosis?
The tuberculosis destroys the subcutaneous tissue
faster than it destroys the skin. The overhanging
skin in this case is blue and unhealthy.
382
Clinical Surgery Pearls
Q 16. What are the differences between floor
and base?
• The floor is what you see and the base is what
you feel
• The floor is the exposed surface of the ulcer.
• The base is on which the ulcer rests
• Pick up the ulcer between the thumb and index
finger for feeling the base
• Slight induration is seen normally for any ulcer
• Marked induration is a feature ofsquamous cell
carcinoma.
Q 17. What are the classical sites for the commonly
seen ulcers?
Classical sites for commonly seen ulcers
Type of ulcer Site
Venous ulcer Just above the medial malleolus
(Gaiter area)
Arterial ulcer Tips of toes and between the toes
(where the pressure is lowest), over
the malleoli and heel (pressure areas)
Neuropathic
ulcer
Over the heads of the first and second
metatarsal.
Traumatic
ulcer
(footballer’s
ulcer)
Shin (the tibia is subcutaneous and
there is lack of underlying muscle with
resultant reduced blood supply)
Rodent ulcer
(BCC)
Above a line joining the angle of the
mouth to the lobule of the ear
Tuberculous
ulcer
Seen in neck, axilla and groin (where
tuberculous lymph nodes are seen)
Gummatous
ulcer
Over the subcutaneous bones such as
sternum, skull, tibia, etc.
Trophic ulcers On the heel and ball of the foot.
Q18. What are the stigmata of Hansen’s disease?
Stigmata of Hansen’s disease
There are three types of leprosy—the lepromatous,
tuberculoid and mixed
Lepromatous leprosy
• Leonine facies (subcutaneous tissue becomes
infiltrated with granulomatous masses)
• Loss of outer half of the eyebrow hair
• Destructionofnasalcartilages—saddlenosedeformity
• Testicular atrophy
• Gynecomastia
Tuberculoid
• Nerve paralysis
• Look for tender thickening of the following nerves
– Ulnar nerve at the elbow
– Great auricular nerve in the posterior triangle
below the ear
– Lateral popliteal nerve around the neck of fibula
• Ulnar claw hand
• Claw foot
• Trophic ulcers.
Q 19. What are the stigmata of syphilis?
Syphilitic stigmata
• Alopecia (loss of hair)
• Bossing of the skull
• Depression of the bridge of the nose
• Interstitial keratitis
• Otitis interna
• Perforation of the nasal septum
• Perforation of the hard palate
• Chronic superficial glossitis
• Hutchinson’s teeth
• Mucous patches
• Condylomas
• Enlarged occipital lymph nodes
• Enlarged epitrochlear lymph nodes
• Gummatous orchitis
• Clutton’s joints
• Sabre tibia.
Ulcer
383
Q 20. What is the peculiarity of venous ulcer in
relation to the depth of penetration?
The venous ulcers usually do not extend beyond
the deep fascia, unlike ischemic ulcers, which
destroys the deep fascia and exposes the tendons,
bones and joints.
Q 21. What are the types of discharges from the ulcer?
Types of discharges and the probable causes
Discharge Cause
• Serous discharge In healing ulcer
• Serosanguinous discharge Tuberculous ulcer
• Greenish/bluish pseudomonas Ulcer infected with
•Yellowdischarge andcreamypus Staphylococci
• Watery and opalescent Streptococcus
• Yellow granules Actinomycosis
Q 22. What is the significance of granulation
tissue?
Granulation tissue signifies healing. Itis usually pink
with red dots. The red dots are seen at the sites of
capillary loops.
Q 23. What is the significance of bluish granulation
tissue in the floor?
I t is suggestive of tuberculosis (Apple-jelly
granulation).
Q 24. What is Wash-leather appearance (wet
chamois leather)?
It is suggestive of syphilitic ulcer—gummatous
ulcer.
Q 25. If the floor is covered with black mass what
is the inference?
It is suggestive of malignant melanoma.
Q26. What is neurotrophic ulcer (perforating)?
Neurotrophic ulcers are seen in the following
conditions:
Causes for neurotrophic ulcer
1. Diabetic neuropathy (peripheral neuritis)
2. Transverse myelitis
3. Syringomyelia
4. Tabes dorsalis
5. Spina bifida
6. Injury to spinal cord
7. Sciatic nerve injury
8. Hansen’s disease (Leprosy).
Q 27. What are the characteristic features of
tuberculous ulcer?
The tuberculous ulcer results as a result of bursting
of caseous lymph node. It is seen in places where
tuberculous lymph nodes are seen-neck, axilla
and groin.
The characteristic features are as follows:
Features of tuberculous ulcer
1. Undermined edge
2. Sites—axilla, neck and groin (where tuberculous
nodes are seen)
3. Floor is covered with blue granulation tissue (Apple
- jelly granulation)
4. Serosanguinous discharge from the ulcer.
Q 28. What is lupus vulgaris?
Itis nothing but cutaneoustuberculosisseen in the
face and hand. The ulcer heels at the center and
spreads at the periphery (like a wolf) and hence
the name lupus means wolf.
384
Clinical Surgery Pearls
Q 29. What are the characteristic features of
venous ulcer (Named by John Gay in 1867)?
• TheyareseenintheGaiter area of the leg (between
the two malleoli and the tibial tuberosity)—
usually above and behind the medial malleolus
• The ulcer is painless
• Ulcer never penetrates the deep fascia
• Surrounding skin will be pigmented and
thickened (lipodermatosclerosis)
• One or more large feeding veins can be seen
proceeding towards the edge of the ulcer
• Varicoseveinsaffectingthelongsaphenous,short
saphenous and perforating veins will be visible.
Q 30. What are the precursors of venous ulcer?
• Dermatitis (eczema)
• Pigmentation (sign of venous stasis)
• Splay of venulesfrom the medial malleolus(flare
sign).
Q 31. What is post-thrombotic ulcer?
Venous ulcer may be secondary to deep vein
thrombosis and subsequent valvular incompetence
resulting in chronic venous hypertension or it may be
secondary to the superficial system incompetence.
When it is secondary to deep vein thrombosis, it is
called post-thrombotic ulcer. The peculiarity of this
ulcer is it will be painful. In such cases, varicose
veins are lacking in spite of careful search. Extensive
induration is a remarkable feature in this case.
The skin appears to be tethered to the underlined
structures, which may extend half way up of the calf.
Q 32. What is gravitational ulcer?
It is another name for venous ulcer.
Q 33. What are the characteristic features of
arterial ulcer?
• They are usually seen at the tips of toes and
between the toes (heel and malleoli).
• Seen in usually older age group
• Destroysthedeepfascia andexpose the tendons
• Ulcers are punched out
• Peripheral pulsations will be absent.
Q 34. What is Meleney’s ulcer?
Itis alsocalledsynergistic ulcer (symbiotic). Itisdue
to symbiotic action of microaerophilic nonhemolytic
streptococci and hemolytic Staphylococcus aureus.
This was originally described in relation to the
infected abdominal and thoracic operation
wounds. However, it can occur in the leg and
hands, arising either de novo are as a complication
of preexisting ulcers. The characteristic feature of
the ulcer is burrowing with a resultant undermined
edge, which may extend for 2 cm. The ulcer is painful
and tender and shows a tendency to spread. There
will be a central purplish zone surrounded by red
inflammation initially. This purplish zone becomes
gangrenous producing an ulcer.
Q 35. What is factitious ulcer (Artefact ulcer)?
It is also called automutilation ulcer. This is a selfinduced ulcer of the leg seen in highly neurotic
individuals or in a litigant desirous of obtaining
compensation. The mode of producing the ulcer
varies. The ulcer is situated always in accessible
places like anterior and lateral surface of the leg.
Usually the ulcer will have clean pink healthy look
with unusual shapes. If the ulcer is covered with,
plaster cast so that the wound cannot be tampered
the wound will heal.
Q 36. What is the feature of ulcer associated with
Erythrocyanosis frigida (Bazin’s Disease)?
This is usually seen in young women with plump legs
and thick ankles living in cold climates. The patients
are troubled by chilblains. Small superficial painful
nodules will be felt in the legs, which are areas of fat
Ulcer
385
necrosis, which will breakdown to form the ulcers. In
addition, the blood supply to the lower third of the leg
will be diminished producing ischemia of the leg. The
skin is abnormally sensitive to temperature changes.
Q 37. What is the cause for ulcer in rheumatoid
arthritis?
This is seen in 20% of the patients. It is due to
breakdown of a nodule. The ulcers may be more
than one having punched out edge seen in the
lateral surface of the lower third of the leg.
Q 38. What is the situation of the ulcer secondary
to osteitis deformans (Paget’s disease)?
These small deep ulcers are situated right over the
convexity of the anteriorly bowed tibia. The base
of the ulcer is bone and the edges are densely
adherent to the bone.
Q 39. What is tropical ulcer?
This ulcer is due to infection by Vincent’s organism
(Bacteroides fusiformis) together with many
pyogenic bacteria, secondary to trauma or insect
bite. It commences as a papulo pustule which in a
matter of hours becomes surrounded by a zone of
inflammation and induration. This is accompanied
by tender lymphadenitis. In two or three days
the pustule bursts and ulcer forms. The edges are
undermined. There is copious serosanguinous
discharge. The ulcer will remain indolent for a long
time. Pain is a constant feature.
The classical features are:
• Profuse serosanguinous discharge
• Over powering vile odor
• Unremitting pain
• Minimal constitutional symptoms
• Extreme tenacity of the slough.
On healing it leaves a permanent scar which is
circular, parchment like and faintly pigmented.
Q 40. What is the feature of ulcer due to Yaws?
The causative organism is Treponema pertenue. The
primary sore may be found on the legs or foot or
buttocks (of children before they walk). They are
painless and in the course of healing form tissue
paper like scars. In the tertiary stage multiple deep
ulcers are seen.
Q 41. What is diphtheritic desert sore?
As the name implies it is seen in the desert.
The organism responsible is Corynebacterium
diphtheriae. It begins as a papulo-pustule. Within
a few days it will form an ulcer reaching the size of
1–2 cm. The floor will be covered by diphtheritic
membrane, which will be difficult to remove. Rarely
the patient shows signs of peripheral neuritis due
to toxins produce by diphtheria.
Q 42. What is Martorell’s ulcer (hypertensive
ulcer)?
This is seen in old age group with atherosclerosis.
A patch of skin on the outer side of the calf suddenly
becomes gangrenous and sloughs away producing
a punched out ulcer. Even though it is an ischemic
ulcer all the peripheral pulses will be normal.
Q 43. What is decubitus ulcer?
It is a synonym for bedsore. It is a type of direct
traumatic gangrene. The bedsores are predisposed
by factors like:
• Pressure
• Injury
• Moisture
• Anemia
• Malnutrition.
They typically appear over areas subjected to
pressure like sacrum, gluteal region and heel in
bedridden patients.
386
Clinical Surgery Pearls
Q 44. What is the warning signal for bedsore?
The bedsore is to be expected over an erythema,
which does not change color on pressure.
Q 45. How will you prevent bed sore?
Prevention of bedsore
• Skilled nursing
• Frequent change of position (2 hourly change of
posture)
• Use of adhesive films such as Opsite
• Water bed—ripple bed
• Keep the area dry.
Q 46. Is there an entity called diabetic ulcer?
No diabetic ulcers are of multiple etiologies and
therefore it is important to identify the cause of
the ulcer in diabetes. The various causes for ulcer
formation in diabetes mellitus are:
1. Arterial
– Atherosclerosis (10 years earlier than the
normal population)
– Microvascular.
2. Neuropathy
– Sensory—glove and stocking type of
peripheral neuropathy
– Motor (Intrinsic muscle paralysis with
resultant unopposed action of long flexor
tendons → shortening of the longitudinal
arches → heads of metatarsals are subjected
to additional load during walking).
– Autonomic—Dry skin due to the absence of
sweating
3. Deformity in diabetic foot.
Therefore in a given case the ulcer may be purely
neuropathic or arterial or it may be a combination
of factors, which the student has to identify.
Q 47. What is the commonest site for neuropathic
ulcer in diabetes?
Over the heads of the first and second metatarsals.
Q 48. What is Marjolin’s ulcer?
It’s a malignant ulcer developing in burns scar or
venous ulcer or edge of any chronic ulcer or chronic
discharging sinuses such as osteomyelitis is called
Marjolin’s ulcer. Malignant change can occur in
the tuberculousskin scarring of lupus vulgaris. It is
nothing but a squamous cell carcinoma of skin with
everted edges or rolled out edges. These changes
can be easily missed and the clinician must always
be on the look out for any changes in the edge.
The peculiarity of this malignancy is the absence of
involvement of regional lymph nodes. The scarring
process in this condition destroys the lymphatics in
the ulcer area.
Q 49. What is the management of the ulcer?
The principles of management of the ulcer are as
follows:
1. Identify and correct the comorbid factors like
DM, anemia, leukemia, etc. by doing:
• FBS—If diabetic, control the diabetes
• PPBS
• Peripheral smear
• Hemoglobin
• Total leukocyte count
• X-ray chest—to rule out tuberculosis.
2. Determine the etiology of the ulcer:
• Biopsy from the edge of ulcer
• If pus is present, send for culture and
sensitivity and give appropriate antibiotic
• X-ray of the part to rule out osteomyelitis.
3. Adequate drainage and de sloughing
Ulcer
387
4. Care of the ulcer:
• Daily dressing
• Avoid antiseptic solutions—impair capillary
circulation
• Use normal saline for cleaning
• Use nonadherent and nonallergic dressing
• Use microporous polyurethane film which
are permeable to gases and water and
impermeable to microorganisms.
5. Treat the underlying cause:
• For example, Antituberculous drugs in the
case of tuberculous ulcer
• Treatthe varicose veinsin the case of venous
ulcer
• Revascularization in the case of arterial ulcers.
6. Excision of the ulcer and skin grafting: If the
ulcer is not healing.
37 Malignant Melanoma
Case
Case Capsule
A 65-year-old male patient presenting with an
ulcer over the right heel of size 5 cm diameter of 8
months duration. On examination, the floor of the
ulcer is covered with black-colored granulation
tissue. The ulcer is not mobile and there is fixity
to the deeper structures. The vertical group of
inguinal nodes are enlarged, firm in consistency,
discrete and mobile. Two horizontal group (medial)
of nodes are also enlarged, which are firm discrete
and mobile. Systemic examination is normal.
‘Beware of the patient with a glass eye, and missing toe’
Read the checklist for examination of the ulcer.
Checklist for history
1. Family history of malignant melanoma
2. History of risk factors—Sun exposure
3. History of nonmelanoma skin cancer
4. History of change in size, shape, color,
inflammation, crusting, bleeding, etc. of the mole
5. History of increase in size
6. History of enucleation of the eye and operations
on the skin.
Checklist for examination
1. Look for the ABCDE of melanoma that is mentioned
below
2. Look for satellite nodules and intransit metastasis
3. Look for regional lymph nodes
4. Look for missing toes and glass eyes
5. Intense search is made for the primary when the
patient is presenting with metastatic nodes
6. Examine the abdomen to rule out hepatomegaly
7. Examine the chest for evidence of metastasis.
Malignant Melanoma
389
Q 1. What is your diagnosis?
Malignant melanoma.
Q 2. Why malignant melanoma?
An ulcer with black pigmented floor and enlarged
inguinal nodes is in favor of diagnosis of malignant
melanoma.
Q 3. What is malignant melanoma?
It is a skin neoplasm arising from melanocytes, a
cell of neural origin.
Q 4. What are the differential diagnoses?
Differential diagnoses for malignant melanoma
1. Pigmented basal cell carcinoma
2. Junctional nevus
3. Seborrheic warts
4. Thrombosedangioma
5. Hemangioma
6. Telangiectasis
7. Granuloma.
Q 5. What are the organs where melanoma can
occur?
Organs involved in malignant melanoma
• Skin
• Eye:
a. Uveal tract – Iris
– Ciliary body
– Choroids
b. Retina
• Meninges
• Mucocutaneous junction
• Conjunctiva.
Q 6. What are the modes of spread of malignant
melanoma?
Modes of spread of malignant melanoma
1. Direct extension
2. Lymphatic—embolism and permeation
3. Hematogenous
– Lungs
– Liver
Contd...
390
Clinical Surgery Pearls
Q 14. What is melanoblast? (PG)
They are believed to originate in the neural crest.
They have the power of forming the pigment and
they are seen in the basal layer of epidermis. The
cells are Dopa positive and Fontana positive.
Q 15. What are melanophores? (PG)
They are dermal macrophages carrying the pigment.
They are Dopa negative and Fontana positive.
Q 16. What is the pathology of a mature adult
mole? (Intradermal mole)
They consist of clusters of melanocytes in the
dermis and therefore, they are called intradermal
mole. Macroscopically the mole can be flat or
raised, smooth or warty, hairy or nonhairy. They
hardly ever turn malignant.
Q 17. What is a junctional mole?
If the movement of the melanocytes stop before
they have all migrated into the dermis, there will
be clusters of cells at various stages of maturity
in the epidermis and dermis. This lesion is called
junctionalmole.Junctional moles are immature and
unstable and can turn malignant. The majority of
themalignantmelanomasbegininjunctionalmoles.
Q 18. In which part of the skin you get junctional
moles?
• Palms of the hands
• Soles of the feet
• External genitalia.
Note: Higher incidence of malignant melanoma is
seen in these sites.
Q 19. What is a compound mole?
When intradermal and junctional features are both
present in one mole, it is called compound mole.
Q 20. What is juvenile mole (spitz nevus)?
Amole showingjunctional activitybeforepuberty is
– Brain
– Bones
– Intestines
– Breast.
Q 7. What is the spread of malignant melanoma
of the uveal tract?
There are no lymphatics in the uveal tract. They
rarely metastasize to lymph nodes.
Q 8. What is a nevus?
The word nevus means a lesion that is present
since birth.
Q 9. What is a mole?
Benign melanin producing lesions are called moles.
Q 10. What is the average number of moles a
person will have?
About 80–100 moles in most Caucasians.
Q 11. In which area of the body moles are commonly
seen?
They are most common in limbs, face and
mucocutaneous junctions (the mouth and anus).
Q 12. What are the layers of the skin?
The skin has got epidermis and dermis.
The epidermis has got five layers.
The five layers of the epidermis are:
• Stratum corneum
• Stratum lucidum
• Stratum granulosum
• Stratum spinosum (prickle cell layer)
• Stratum basale (Basal layer).
Q 13. In which layer of the skin the melanocytes
are seen?
They are normally found in small numbers among
the cells of the basal layer of epidermis.
Contd...
Malignant Melanoma
391
called juvenile mole (pigmented spitz nevus). They
finally become mature intradermal moles.
Q 21. What is a blue nevus?
When the melanocytes migrate to the bottom of the
dermis and into the subcutaneous tissue, the lesion
will get a blue appearance and it is called a blue nevus.
Q 22. In which ethnic group malignant melanoma
is more common?
It is more common in Caucasians living in hot countries
such as Australia (because of the greater quantity of UV
light exposure). It is less common in Negroes.
Q 23. What is the incidence of malignant melanoma?
• The incidence of malignant melanoma is rising
• It forms 3% of all malignancies
• It forms 5% of the cutaneous malignancy
• 7% present as occult metastasis
• 10 – 20% occur in pre-existing nevi
• Seen 20 times more in whites
• Lowest incidence in Asia
• The incidence has doubled in Britain, Norway,
Canada and America
• The incidence has quadrupled in Australia.
Q 24. What are the types of malignant melanoma?
Types of malignant melanoma
• Superficial spreading
• Nodular
• Lentigo malignant
• Acrolentigenous
• Amelanotic.
Q 25. What are the growth phases of malignant
melanomatous lesions?
It has two types of growth:
1. Radial growth phase, e.g. the superficial spreading
type (all melanomas show radial growth phase
except nodular melanoma. Radial growth is an
intraepidermal growth).
2. Vertical growth phase, e.g. the nodular melanoma
(The vertical growth is the tumor growth in
dermis leading to nodule formation).
Q 26. What are the features of superficial spreading
type of malignant melanoma?
• It forms 70% of the lesions
• Peak incidence in the 5th decade
• Common in legs and back
• Long radial growth phase
• May arise in pre-existing nevus
• Wood’s lamp can identify radial growth phase
• Darkbrown,blue,black,pinkorgray,whiteincolor.
Q 27. What are the features of nodular melanoma?
• It is the most malignant type of melanoma
• Forms 15%ofthe lesions,twice common in men
• Predominant growth phase is vertical
• Common in trunk, head and neck
• Twice common in men
• Ulceration of the lesion is seen
• Crust formation also seen.
Q 28. What are the features of lentigo malignant
melanoma?
• It forms 5–10% of the lesions
• Itis aninsituvariantwithlessmetastaticpotential
• It arises in Hutchinson’s melanotic freckle
• Seen in old age (women more)
• Seeninsunexposedskin(prolongedandintense
exposure)
• Lesions are mainly seen in head and neck
• 5 mm margin is enough for excision.
Q 29. What is Hutchinson’s lentigo?
1. This is a term used to describe a large area of dark
pigmentation, seen commonly on the face and
neckinlateadultlife.Ithasgottwospecialfeatures:
392
Clinical Surgery Pearls
2. Late development
3. High incidence of malignant change.
Some pathologists consider this as precancerous. The surface is smooth with some raised
areas suggesting the sites of junctional activity.
Q 30. What is Hutchinson’s halo?
• This is a halo of brown pigment in the skin
around the melanoma and satellite nodules. The
halo is suggestive of malignant change in a preexisting mole.
Q 31. What are the features of acral lentiginous?
• It affectsthe palms,soles, and beneath the nails
(subungual)
• More seen in dark skinned group—70% of
melanomasin black, 46% in Asians(It will affect
any ethnic group)
• No history of sun exposure
• It forms 2–8% of the melanomas in white
• 46% of melanomas in Asians
• Usually > 3 cm in size
• Late presentation is a common feature
• The melanoma is more aggressive.
Q 32. Why the amelanotic melanoma is called so?
About 25% are amelanotic.
Here the malignant melanomatous lesion is not
black and they appear as white. Therefore, it is called
amelanotic melanoma.
Q 33. What are the features of amelanotic
melanoma?
• The prognosis ofthistype is worse than nodular
melanoma
• The lesions appear pink
• There will be delay in the diagnosis
• In GI tract it will produce obstruction.
Q 34. What is the ABCDE system for evaluation of
pigmented skin lesion?
Look for the following things in a pre-existing
pigmented lesion. If they are present it is suggestive
of malignancy.
ABCDE system for evaluation of malignant
change in a pigmented lesion
A – Asymmetry
B – Border irregularity
C – Color – variegation
D – Diameter > 6 mm
E – Elevation
Q 35. What is the Glasgow seven point checklist?
Glasgow seven point checklist
1. Change in size
2. Change in shape
3. Change in color
4. Inflammation
5. Crusting and bleeding
6. Sensory change
7. Diameter > 6 mm.
Q 36. What is satellite lesion?
Intralymphatic metastatic lesion within 2 cm of
the primary tumor is called satellite lesion. This is
considered as an extension of the primary and is
categorized as N2C (stage III C) in the new AJCC
7th edition.
Q 37. What is intransit metastasis?
Intralymphatic metastasis more than 2 cm from the
primary lesion but not beyond regional lymph node
basin is called intransit metastasis (N2
C).
Q 38. What are the risk factors incriminated in
malignant melanoma?
The various risk factors are:
Malignant Melanoma
393
1. Sun exposure—most common (50%)—sun
exposure before the age of 10 years and
significant adult exposure
2. Multiple atypical nevi
3. Multiple benign nevi > 100
4. GCPN—Giant congenital pigmented nevus –
3–5% life time risk for malignant melanoma
5. Dysplastic nevus syndrome
6. Xeroderma pigmentosum
7. Nonmelanoma skin cancer (NMSC)
8. Previous malignant melanoma skin cancer
9. Immunosuppression (HIV, Hodgkin’s)
10. Use of tanning lamps/photochemotherapy
11. Familyhistoryofmalignantmelanoma (8–12%)
12. Red hair
13. Tendency to freckle.
Q 39. What is the role of heredity in malignant
melanoma?
• Familial malignant melanoma
• When three first degree relatives are affected it
should be suspected
• There will be mutation in CDKN2A gene
• Genetic predisposition
• 8 – 12% of all melanomas are hereditary
• Second primary will be seen in 3–5% of cases.
Q 40. What is Breslow’s thickness?
The thickness of the melanomatous lesion is
measured from the top of the granular cell layer,
to the base of the tumor with the help of ocular
micrometer.Thisis calledBreslow’sthickness,thatis
the most important prognostic factor for malignant
melanoma. The original dimensions described by
Breslow are not considered now for classification.
Q 41. What is a thin melanoma?
A melanoma that is less than 1 mm is called thin
melanoma. The survival of patients with this lesion
is 95%.
Q 42. What is intermediate thickness lesion?
Lesions of thickness between 1 – 4 mm are called
intermediate thick lesions.
Q 43. What is thick melanoma?
Lesion with thickness more than 4 mm is called
thick melanoma.
Q 44. What are the bad prognostic factors for
melanoma? (PG)
The bad prognostic factors are:
Bad prognostic factors
1. Tumor thickness in mm is the most important
prognostic indicator
2. LDH
3. Mitotic rate
4. Tumor infiltrating lymphocytes (TIL)
5. Ulceration—more than 3 mm ulcer
6. Vertical growth phase
7. Regression.
Q 45. What is the new AJCC staging system for
malignant melanoma (7th edition)? (PG)
The summary of changes in 7th edition are:
• Mitotic rate is an important primary tumor
prognostic factor and replaces level of invasion
• Nodal micrometastasis can be defined by H & E
or immunohistochemical staging
• Metastatic melanoma in LN, skin, and s/c tissue
from an unknown primary site is categorized as
stage III rather than stage IV
• Sentinal LN biopsy is recommended for
indentifying occult stage III disease in patients
with clinical stage IB or II melanomas
• Thickness and ulceration in contrast to level of
invasion used in T-category are still used
• Satellite mets and intransit metastasis grouped
into stage IIIc disease.
394
Clinical Surgery Pearls
T–classification
TX: Primary tumor cannot be assessed (e.g. curettaged or severely regressed melanoma)
T0: No evidence of primary tumor
Tis: Melanoma in situ.
T
Classification
Thickness
(mm)
Ulceration status/mitoses
T1 Tumor <
1 mm
a = without ulceration and
mitosis < 1/mm2
b = with ulceration or
mitosis ≥ 1/mm2
T2 Tumor 1
– 2 mm
a = without ulceration
b = with ulceration
T3 Tumor
2 – 4 mm
a = without ulceration
b = with ulceration
T4 Tumor >
4 mm
a = without ulceration
b = with ulceration
Note:
• Ulceration is defined pathologically asfullthickness
epidermal defect including absence of stratum
corneum and basement membrane
• 1 mm2 is approximately 4 high power fields at 400
X in most microscopes.
Regional lymph nodes
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Metastasis in one lymph node (a) micrometastasis, (b) macrometastasis
N2 Metastasis in two to three regional nodes or
intralymphatic regional metastasis without
nodal metastases (a) micrometastasis, (b)
macrometastasis, (c) in transit mets/sat mets
without metastatic LN
N3 Metastasis in four or more regional nodes,
or matted metastatic nodes, or intransit
metastasis or satellite (s) with metastasis
in regional node
Distal metastasis (M)
M0 No distant metastasis
M1 Distant metastasis
M1a Metastasis to distant skin, subcutaneous
tissues or distant lymph node metastasis with
normal LDH
M1b Metastasis to lung with normal LDH
M1c Metastasis to all other visceral sites or distant
metastasis to any site combined with an
elevated serum lactic dehydrogenase
(LDH).
Clinical stage grouping (used after complete excision
of the primary melanoma with clinical assessment
for regional and distant metastasis).
Stage Tumor size N M
0 Tis N0 M0
IA T1a N0 M0
IB T1b N0 M0
T2a N0 M0
IIA T2b N0 M0
T3a N0 M0
IIB T3b N0 M0
T4a N0 M0
IIC T4b N0 M0
IIIA Any T 1 – 3 lymph nodes with
microscopic metastasis Primary melanoma not ulcerated
IIIB Any T 1 – 3 lymph nodes with
macroscopic mets and nonulcerated primary
or
1 – 3 microscopic lymph
nodes with ulceration
or
intralymphatic regional mets
without nodal mets
Contd...
Malignant Melanoma
395
IIIC Any T 1 – 3 macroscopic lymph
nodes + ulcerated primary
melanoma
or
Patientswithsatellite/intransit
of ulcerated melanoma
or
Any patient with N3 regardless of T status
IV Any T Any N M1
Q 46. What is Clark’s level?
This is used for the pathological assessment of the
depth of invasion of the malignant melanoma.
Because of the discrepancy in assessment of the
pathological depth amongpathologistsClark’slevel
is used only for T1 lesions.
Clark’s levels for depth of invasion
Level I Lesion confined to the epidermis
Level II Lesion extending to the papillary dermis
Level III Lesion filling the papillary dermis
Level IV Lesion involving the reticular dermis
Level V Lesion extending to the subcutaneous tissue
Q 47. What is the confirmatory investigation for
the diagnosis of this lesion?
Biopsy.
Q 48. What type of biopsy is recommended and
what are the precautions taken?
• Excision biopsy is the procedure of choice,
elliptical incision is preferred.
• Consider definitive therapy before choosing
technique
• 1 to 3 mm margin preferred
• Avoid wider margin to permit accurate subsequent lymphatic mapping
• Orientationofincisionandspecimenareimportant
• Biopsy up to the subcutaneous tissue
• There is no role for shave biopsies
• Incisionbiopsy isdoneonly forlargelesions—The
incision biopsy may be taken from the thickest
part as the last resort. It will interfere with the
accurate Breslow’s thickness determination that
decides the ultimate excision margin (some areas
may be thicker than others and therefore, full
histological examination is not possible). It is also
done for lesions of the face and ear
• Thickness of the lesion should be reported
• The deep fascia is not excised, unless involved
• In other placesit is better to consider a splitskin
graft or fasciocutaneous flap.
Note: Original excision margin recommended by
Handley in 1907 was 5 cm, that is having no clinical
evidence support.
Q 49. What are the current guideline for the
margin of excision?
• In situ – 0.5 cm
• Less than 1 mm thickness – 1 cm margin and
lesion primary closure
• 1 – 2 mm thickness lesion – 2 cm margin is
recommended
• More than 2 mm – 2 cm margin
thickness lesion
Q 50. What is the margin of excision recommended
for subungual melanoma?
1 cm margin is not possible in this situation
without excision of the terminal phalanx. In general
amputation of the digit is recommended.
Q 51. What are the diagnostic immunohistochemical markers for malignant melanoma? (PG)
Immunohistochemistry must include at least one
melanoma specific marker. They are:
Contd...
396
Clinical Surgery Pearls
• HMB—45 (Premelanosomal protein)
• Melan—A
• Mart 1
Q 52. What is the management of lymph node?
• If clinically enlarged FNAC is preferable to
excision biopsy for confirmation
• Open biopsy may increase the risk of tumor
spillage
• If open biopsy is required the incision should
be placed in such a way that it can be excised in
continuity with lymph node field.
Q 53. What is the management of positive node?
• Therapeutic radical lymph node dissection is
recommended
• For the lower limb it is radical ilioinguinal block
dissection
• Limited dissections such as superficial femoral
dissection and node picking are not recommended.
Q 54. What is the role of elective lymph node
dissection—ELND (means managing the nodes,
which are not involved)? (PG)
• Only 25% of the patients will show histological
occult metastasis in such dissection
• It is unnecessary, therefore, in 75% of patients
• There is no survival advantage for ELND
• Thus, it is not recommended routinely.
The arguments in favor of ELND are:
• Incidence of positive nodes is 25–65%
• Survival rate are lower in patients who develop
nodes
• The recurrence rate is higherwhen there is extracapsular spread or multiple nodes
• Patient may fail to attend regular follow-up
• The nodedissection carries negligible morbidity.
Q 55. What is sentinel lymph node biopsy? (PG)
Indications are T1
b, T2
, T3
and T4
melanoma with
clinically or radiologically uninvolved lymph nodes.
It is done prior to wide excision. The anatomical
concept is that lymphatics from defined regions
of skin drain specifically to an initial node or nodes
(sentinel nodes) prior to disseminating to other
nodes in the same or nearby basins.
Sentinel lymph node biopsy is a technique
initially described by Cabanas in 1974 for penile
carcinoma. This was popularized by Morton in
1994 for melanoma. The aim of this technique
is to identify those patients in whom it may be
appropriate to carry out elective lymph node
dissection by identifying the micrometastasis in
the sentinel node.
Preoperative method—lymphoscintigraphy
The technique is to identify the first echelon lymph
node (sentinel node) by lymphoscintigraphy
following intradermal injection of radioactive
technetium99 sulfur colloid around the primary
site. A hand held gamma probe helps to locate
the node. The position of this node is marked on
the skin surface.
Perioperative method
The lymph node is identified intra-operatively by
injecting patent blue dye around the site of the
primary lesion.The node isremoved and subjected
for frozen section examination. If the node is found
to be, pathologically positive radical lymph node
dissection is carried out.
Q 56. What are the advantages of sentinel lymph
node biopsy? (PG)
The node is send for frozen section, routine H and E
staining and immunohistochemical staining.
Malignant Melanoma
397
• The pathologist can study 1 to 2 nodes fully
rather than 10 to 30 nodes.
• Routine H and E staining can identify 1 tumor
cell/10,000 normal size.
• Reverse transcriptase PCR will identify tumor
protein production and one tumor cell per
1 million is identifiable.
• Histology negative but PCR positive tumors are
having worse prognosis than histology negative.
• PCR status of the lymph node is the most
significant predictor of survival even over
tumor thickness.
Q 57. What are the investigations for melanoma?
1. Since wide excision is not possible in this case
because of the fixity, take incision biopsy from
the thickest part of the lesion (excision biopsy
is the investigation of choice normally).
2. USG examination of the regional lymph nodes
3. FNAC of lymph node
4. LDH
5. Sentinel lymph node biopsy for stage Ia and II
6. CT/PET/MRI—abdomen/chest/pelvis(stage IIto
IIc) if clinically indicated.
7. Sentinel node positive and clinically stage III
disease—CT/PET/MRI.
Q 58. What is the staging in the given case?
Since there is inguinal lymph node enlargement
it is Stage III.
Q 59. What is the recommended surgical treatment
in the given case?
Since, it is a big ulcer with fixity to the calcaneum
he needs a below knee amputation for the primary
along with ilioinguinal block dissection for the nodes.
Q 60. What is desmoplastic melanoma? (PG)
In desmoplastic melanoma the following features
are seen:
• Histologically spindle cells are seen in fibrotic
stroma with lymphocytic infiltration
• There is high rate of recurrence
• Wider margins are desirable
• Minimum of additional 1 cm margin is
recommended
• They are rare and can arise de novo.
Q 61. What is neurotropic melanoma? (PG)
Neurotropic melanoma is characterized by:
• Peri/Intraneural infiltration
• It forms > 30% of desmoplastic melanoma
• High rate of recurrence
• Additional 1 cm margin is recommended.
Q 62. Any special form of treatment recommended for childhood melanoma? (PG)
• Melanoma in childhood is rare
• The differential diagnosis is pigmented spitz
nevus(juvenile melanoma) that is a compound
naevus in childhood
• Same treatment is recommended as in adults.
Q 63. What is the management of melanoma in
pregnancy? (PG)
• Same treatment as in nonpregnant patient
• Early termination of pregnancy when the
diagnosis is made
• Delay pregnancy for 2 years after treatment for
melanoma
• Placental and fetal metastasis can occur in
advanced melanoma.
Q 64. What is the incidence of mucosal melanomas? (PG)
• It forms < 1% of all melanomas
• Commonest site is oral cavity.
Q 65. What are the locations where you get
mucosal melanomas?
Mucosal melanomas are seen in the following
situations:
398
Clinical Surgery Pearls
Sites of mucosal melanoma
• Oral cavity
• Rectum
• Anal canal
• Female genital tract
• Larynx
• Oropharynx
• Hypopharynx
• Nasopharynx
• Paranasal sinuses
• Esophagus (less common).
Q 66. What are the features of oral melanomas? (PG)
The characteristic features of oral melanoma
• Age incidence is between 40 and 60 years
• 50% are on hard palate
• 25% are on the upper gingiva
• 30%are preceded by an area of hyperpigmentation
• Pigmentation varies black to brown
• Red color indicates nonpigmented melanoma
• It may be flat or raised or nodular, later become
ulcerated and may bleed
• It is notoriousfor rapid growth and poor prognosis
• Destruction of the underlying bone is a feature
• 50%willhavemetastasis atthe timeofpresentation.
Q 67. What is the prognosis of mucosal
melanomas? (PG)
They are having poor prognosis. The five-year
survival rate appears to be about 5%.
Q 68. What is the treatment of mucosal
melanomas? (PG)
• Wide excision and reconstruction followed by
radical dissection of the nodes and radical
radiotherapy
• In the case of anal canal an abdominoperineal
resection is recommended.
Q 69. What is the origin of melanoma in the eye?
It originates from the pigment cells of the choroid,
ciliary body or iris.
Q 70. What are the clinical presentations of
melanoma of eye?
It can present as:
• Reduction in vision
• Vitreous hemorrhage
• Elevated pigmented lesion in the eye.
Note: The more posterior the lesion in the eye the
more malignant it is likely to be.
Q 71. What is the spread of melanoma of the eye?
The spread is often delayed for many years and
often goes to the liver. Therefore, the aphorism:
‘Beware of the patient with a glass eye, and an
enlarged liver’—There will be history of enucleation
of the eyeball years back for melanomatous lesion
followed by use of glass eyes. The enlarged liver
may be due to metastasis.
Q 72. What is the treatment of melanoma of the eye?
The following options are available:
Treatment options in melanoma of the eye
• Laser coagulation
• Radioactive plaques
• Radiotherapy
• Local excision using hypotensive anesthesia
• Enucleation.
Q 74. What is the investigations of choice for the
diagnosis of melanoma of choroid? (PG)
Ultrasound examination that will show a solid tumor.
Q 73. What is the role of adjuvant chemotherapy
after surgery in melanoma of the skin? (PG)
• It is the standard of care in the US
• In UK and rest of the World close follow-up
followed by clinical trial in selected cases
Malignant Melanoma
399
• At present chemotherapy as an adjuvant
modality is being given for high-risk patients
with no evidence of systemic metastasis on
clinical trial basis.
• Theonlyapprovedagentfortherapyisinterferon
alpha – 2b (considerable toxicity is there).
Q 74. What you mean by high-risk group?
High-risk group consists of stages IIb, IIc and III.
(node positive and thick node negative).
Q 75. What are the other chemotherapeutic
agents used in melanoma?
• Tumor response is < 20%
• Dacarbazine (DTIC) isthe single mostimportant
drug with a response rate of 15–30%
• Temozolamide—oral analog—crosses blood
brain barrier and it is used for cerebral metastasis.
• Combination chemotherapy—CVD regimen
is used usually—cisplatin, vinblastine and
dacarbazine.
• Tamoxifen – Given along with combination
chemotherapy
Q 76. What is the treatment of local recurrence?
(PG)
Wide excision with 2 cm margin is recommended.
Q 77. What is the treatment of intransit metastasis? (PG)
a. If they are few in number surgical excision with
a margin of surrounding normal cutaneous and
subcutaneous tissues (excised en bloc with
primary if possible and closure of the defect
with flap or graft).
b. Intralesional therapy with granulocyte
macrophage colony stimulating factor (GM-CSF)
c. Laser therapy
d. Radiotherapy
e. Hyperthermic isolated limb perfusion.
Q 78. What is hyperthermic isolated limb perfusion? (PG)
It is introduced by Sydney Melanoma Unit in 1993.
Isolating blood circuit to the extremity and
administering chemotherapeutic agent regionally at a concentration of 15–25 times higher
without side effects is called hyperthermic isolated
limb perfusion. A small bore vascular catheter is
introduced into the femoral vessel from contralateral
limb. A pneumatic tourniquet above the limb is
applied and a small bore vascular catheter is used
for introducing the agent.
• MelphalanandActinomycinDin400mLofsaline
• Gives significant palliation of locoregional
symptoms
• No improval in survival is noted.
Q 79. What are the monoclonal antibodies
available for treatment?
• Trametinib
• Dabrafenib.
Q 80. How do you stage metastatic melanoma of
unknown primary?
• It is considered as stage III rather than stage IV
• Visceral metastasis is stage IV
It may be as a result of:
1. Previously biopsied and regressed
2. From ocular primary
3. From mucosal melanoma
• Look for primary
• Do surgery for the metastatic lymph node.
Q 81. Is there any role for radiotherapy in
malignant melanoma?
Even though melanoma was considered to be
resistant to radiotherapy, it is indicated in certain
special situations with the dose mentioned below.
• More than 4 Gy dose-high response rate
400
Clinical Surgery Pearls
• Large facial lentigo malignant melanoma
• Medically inoperable, those refusing surgery,
thick melanoma, desmoplastic melanoma
• Incomplete excision ofregional nodes especially
head and neck
• For metastasis in lung, bone, brain, LN and
subcutaneous nodule
• Lower recurrence rate following adjuvant XRT
(10–25%)
• 50% Response rate for skin lesions and 30% for
brain.
Q 82. What are the sites of metastasis?
• Can metastasize to any organ or site
• Skin, soft tissues
• Lung
• Liver
• Brain
• Bone
• GI tract.
Q 83. What is the management of metastatic
disease?
• Interferon alpha and interleukin 2 are used
• < 5% of patients with metastasissurvive 5 years
(6–10 months)
• Resection of solitary mets can increase 5 years
survival to 30%
• Lung → lobectomy
• Liver → lobar resection
Q 84. What is melanuria? (PG)
• Presence of melanin in urine is called melanuria
• I t is seen in cases of extensive visceral
involvement
• It is a terminal feature of malignant melanoma.
Q 85. What is targeted therapy for malignant
melanoma? (PG)
Two-third of melanomas show activating mutation
in BRAF. There is elevated Raf kinase activity.
Blocking the Raf pathway using BAY 43 – 9006 is
being tried as targeted therapy.
Q 86. What is spontaneous regression?
Spontaneous regression is an immunological
phenomenon. Melanoma is considered an
immunogenic human solid tumor. There will be
no physical evidence of primary in 3 to 15% of
patients and the patients may present with lymph
node metastasis. In such cases, intense search
should be made for the primary. If primary is not
found, treat the metastasis. The regression may be:
• Partial/complete regression
• Presenting as variation in color and irregular
borders
• It is a host antitumor phenomenon
• Antimelanoma antibodies are found in blood
• Regression is associated with risk of metastasis.
Q 87. What are the pathological types of
regression? (PG)
Pathologically the regression may be early,
intermediate or late:
• Early – Lymphocytes are seen disrupting nests
of melanoma cells
• Intermediate – There is loss of continuity of the
lesion with mild fibrosis
• Late – There is extensive horizontal fibrosis.
Q 88. Why there is increased chance for metastasis
in cases of spontaneous regression? (PG)
A subpopulation of melanoma cells escaping
immune recognition is responsible for metastasis.
• Look for primary and excise
• Surgery for the metastatic lymph node.
Malignant Melanoma
401
Q 89. What is melanoma vaccine? (PG)
Unlike other vaccines the melanoma vaccine is used
for treatment and not for prevention.
• Irradiated, allogenic cultured melanoma cells
with or without BCG is used for treatment
(polyvalent melanoma vaccine—3 melanoma
cell line—cancerVax)
• The vaccine isprepared from the melanoma cells
of the patients (autologous tumor vaccine) large
amount of tumor is necessary for the production
of vaccine.
• Vaccines prepared from viruses are also used -
Vaccinia melanoma oncolysates—melacin
• GM2 ganglioside-based vaccine
• DNA immunization.
Q 90. What is the recommended follow-up for
malignant melanoma? (PG)
• Every 3 months for 3 years
• Every 6 months for 2 more years
• Then yearly
• CXR annually.
Q 91. What are the preventive measures for
melanoma?
• Genetic—autosomal dominant mode of transmission
• Sun exposure—intermittent intense exposure
to UV is responsible for melanoma
• Monthly skin self-examination isrecommended
• Clinicalskin examination once or twice a year in
high-risk areas
• Low threshold forsimple excision of pigmented
lesions
• Use of sunscreen.
38 Basal Cell Carcinoma/Rodent Ulcer
Case
Case Capsule
A 60-year-old male patient presenting with
pigmented ulcerated lesion of 2 years duration
at the region of nasolabial fold of the face on the
right side of 1.5 cm size. The ulcer is having raised
and rolled edge which is pigmented. The center of
the ulcer is covered with a scab. The lesion is arising
from the skin and it is mobile. There is no fixity to the
deeper structures. There is no regional lymph node
enlargement. Systemic examination is normal.
Read the checklist for examination of the ulcer.
Read the checklist for examination of melanoma.
Q 1. What is your diagnosis?
Basal cell carcinoma (Rodent ulcer).
Q 2. What is the commonest type of skin malignancy?
Basal cell carcinoma (BCC).
Q 3. Mention one hereditary syndrome associated
with basal cell carcinoma?
Hereditary Gorlin’s syndrome. This syndrome
presents with numerous BCC tumors.
Q 4. What is the origin of basal cell carcinoma?
It is a malignant tumor of pluripotential epithelial
cell arising from basal epidermis and hair follicle—
affecting the pilosebaceous skin.
Q 5. What are the predisposing factors for basal
cell carcinoma?
• UV light (most important)
• Arsenical compounds
• Coal tar
• Aromatic hydrocarbons
• Infrared rays
• Genetic skin cancer syndromes.
Q 6. What is the commonest age group affected?
It is 40–80 years (95% are seen in this age group).
Basal Cell Carcinoma/Rodent Ulcer
403
Q 7. What are the diagnostic points for basal cell
carcinoma?
1. Ulcerated lesion in the rightside ofthe face with
raised and rolled edge
2. Central part of the ulcer is covered with a scab
3. No regional lymph node enlargement.
Q 8. What are the characteristics of BCC?
Characteristics of BCC
1. It grows very slowly (over the course of many years)
2. No lymph node involvement
3. Local destruction by infiltration is the hallmark
4. The edge will be raised and rolled
5. The lesion may be pigmented or nonpigmented
6. It is seen in the classical area above a line joining
the angle of the mouth to the ear lobule.
Q 9. What is the relationship of the BCC to arsenic?
Multiple basal cell carcinomas are seen in persons
who have arsenical dermatitis following the
administration of arsenic.
Q 10. Which sex is affected more by BCC?
• Men more than women
• Fair skinned more than dark skinned.
Q 11. What are the differential diagnoses in this
situation?
1. Malignant melanoma (regional lymph node will
be enlarged)
2. Keratoacanthoma just beginning to slough
3. Squamous cell carcinoma
4. Seborrheic keratosis.
Q 12. What is seborrheic keratosis?
This also called senile wart, senile keratosis, or basal
cell papilloma or verruca senilis or seborrheic wart.
It is a benign over growth of epidermis
containing swollen abnormal epithelial cells which
raise it above the level of the normal epidermis
giving a semitransparent oily appearance. It is seen
on any part of the skin except those areas subjected
to regular abrasions such as palms and sole. The
majority are found on the back of the trunk. They
have a rough and papilliferous surface.
Q 13. What is keratoacanthoma?
• This is a cup-shaped growth
• The central crater is filled with a plug of keratin
• Seen in the face of 50–70-year-old group (more
common in men)
• Papilloma virusinfection,smoking and chemical
carcinogen exposure are etiological factors
• Lesions can grow upto 1 to 3 cm over 6 months
and then, they spontaneously resolve
• Excision is recommended
• Itisalsocalledadenomasebaceumormolluscum
pseudo carcinomatosum.
Q 14. What are the types of basal cell carcinoma?
There are about 26 varieties of basal cell carcinoma.
The important types are:
Important types of basal cell carcinoma
1. Nodular
2. Nodulocystic
3. Ulcerative
4. Morpheic (Sclerosing)
5. Pigmented
6. Superficial
7. Field - fire
8. Cystic
9. Nevoid
10. Infiltrative.
Note: Nodular and nodulocystic variants account
for 90% of BCC.
Q 15. What are the sites for basal cell carcinoma?
They are usually seen on the face above a line drawn
from the angle of the mouth to the ear lobule.
404
Clinical Surgery Pearls
Q 21. What are the bad prognostic types? (PG)
• Infiltrative
• Morpheic.
Q 22. Why morphoeic type spread rapidly?
They synthesis type IV collagenase.
Q 23. What is high risk BCC?
• Lesions larger than 2cm situated near the eye,
nose and ear
• Recurrent tumors
• Presence of immunosuppression.
Q 24. What is the macroscopic classification?
It is divided into:
• Localized (Nodular, nodulocystic, cystic,
pigmented and nevoid)
• Generalized: Superficial (multifocal or superficial
spreading).
Infiltrative (morpheic, ice pick and cicatrizing).
Q 25. What is the microscopic pathology of basal
cell carcinoma? (PG)
• The characteristic finding is ovoid cells in nests
with an outer palisading layer perpendicular
to the surrounding connective tissue. Only the
outer layer of cells will actively divide. This is
the reason for the slower growth rate.
• Mitotic figures are absent.
Q 26. What are the modes of spread of BCC?
• Local infiltration—It is the predominant mode
of spread.
• Perineural invasion is seen in morphea form
of BCC—It is associated with high rate of
recurrence and incomplete excision.
Q 27. What are the options for treatment?
The treatment options are:
1. Surgical excision
2. Destructive Treatments—
a. Electrodesiccation and curettage (EDC)
• Medial canthus of the eye
• Lateral canthus of the eye
• Nasolabial fold
• Nose
• Eyelid
• Cheek
• Ear.
It can also occur in other sites like scalp, neck,
arms and hands.
Q16. Can you get multiple basal cell carcinomas?
Yes.
Q 17. What is the usual evolution of a basal cell
carcinoma (BCC)?
• Usually it will start as a small nodule.
• The center of the nodule will die resulting in
ulcer formation with rolled edge.
• If the center does not necrose and ulcerate the
nodule will look cystic (but actually it will be solid).
Q 18. Why it is called rodent ulcer?
The main mode of spread is local infiltration. The
longstanding ulcers erode deep into the face
destroying the bone and exposing the nasal cavity,
nasal sinuses, the eye and even the brain. Because
the ulcer is eroding into deeper structures like a
rodent, it is called a rodent ulcer. The BCC will kill
the patient by local infiltration.
Q 19. What is geographical type of BCC?
The central area of the lesion is healing and the
peripheral area is spreading and advancing. This
is called the geographical type of BCC or a Forest
Fire type. The edge will be irregular and rolled out
with a white scar in the center.
Q 20. What is pearly edge?
When the nodule is getting ulcerated, the edge
will appear as pearly white nodules just below the
epidermis and therefore it is called pearly edge.
Basal Cell Carcinoma/Rodent Ulcer
405
b. Cryosurgery
c. Carbon dioxide laser
d. Radiotherapy.
Q 28. What is the margin of excision recommended?
Most authors currently recommend a margin of
2– 15 mm depending on macroscopic variant.
Q 29. What is Mohs micrographic surgery? (PG)
This involves:
• Excision of all visible tumors in horizontal slices
while mapping the exact size and shape of the
lesion
• Horizontal frozen sections are taken from the
under surface of the excised lesion
• They are examined microscopically
• Incompletely excisedareas oftumor aremapped
and marked for further excision
• This processisrepeated untilthe entire tumoris
removed.
Q 30. What are the advantages of this technique? (PG)
• High cure rate
• Decreased morbidity
• Tissue conservation.
Q 31. What are the disadvantages of this
technique? (PG)
The only disadvantages is the need for two separate
procedures when reconstruction is required.
Q 32. What is electrodesiccation and curettage?
(PG)
• This form of treatment is effective only for very
small and superficial tumors
• Lesionslessthan 2 mm are completely removed
in 100%
• Lesions 2–5 mm are removed in 85% of cases
• Tumors more than 3 cm recur in 50% of cases.
Q 33. What is the role of cryosurgery?
This is also a form of treatment for small tumors.
Q 34. What are the complications of cryosurgery?
(PG)
• Marked edema
• Permanent hypopigmentation
• Increased morbidity.
Q 35. What is the role of carbon dioxide laser?
• Useful for treatment of multiple tumors
• Useful in patients with hereditary Gorlin’s
syndrome
• Useful only for superficial BCCs confined to the
epidermis and papillary dermis
• Treatment of deeper lesions will produce
scarring
• Laser producessuperficial vaporization oftissue
• Usually requires 3 passes on clinically visible
tumor.
Q 36. What is the role of radiotherapy?
The BCC is radiosensitive with overall cure rate of
92%. The advantages of radiotherapy are:
• Can be used in areas which are difficult to
reconstruct, e.g. eyelids, tear ducts, nasal tip, etc.
• Less traumatic than surgical excision
• No need for hospitalization
• Wide margin of tissue can be treated.
Q 37. What are the disadvantages of radiotherapy? (PG)
• Expensive facilities are necessary
• Radiation dermatitis
• Osteitis and chondritis
• Scarring
• Ulceration
• Ectropion
406
Clinical Surgery Pearls
• Epilation
• Repeated is treatments are required over a
period of 4–6 weeks
• Usually not used in age group less than 40
• Cannotbeusedifnotrespondingtoradiotherapy.
Radiation is therefore used only for elderly
patients who are not suitable candidates for
surgery.
Q 38. What is the role of topical agents?
5FU and imiquimod are used as topical agents in
elderly who are reluctant to undergo surgery.
Q 39. What is the cause of death in BCC?
• Direct intracranial extension by infiltration
• Erosion of major blood vessels.
Q 40. What is the treatment of recurrence?
Immediate re-excision of all lesions.
39 Squamous Cell
Carcinoma—SCC (Epithelioma)
Case
Case Capsule
A 65-year-old male patient presents with ulceroproliferative lesion of 4 cm size on the dorsum of
the left foot of 8 months duration. He is a farmer by
profession. The lateral half of the lesion is ulcerated
having everted edge. The medial half of the lesion
is showing cauliflower-like proliferation. There
is copious, purulent and bloody foul smelling
discharge from the ulcerated region. The floor of
the ulcer is showing unhealthy granulation tissue.
The base is indurated; however, the ulcer is mobile
with no fixity to the underlying structures. The
ulcer bleeds to touch. There are three discrete,
mobile and firm lymph nodes in the vertical group
of inguinal lymph nodes. The examination of the
abdomen and chest are normal.
Read the checklist for examination of the ulcer.
Read the checklist for examination of melanoma.
408
Clinical Surgery Pearls
Q 1. What is the probable diagnosis in this case?
Squamous cell carcinoma (SCC) of the dorsum of
the foot.
Q 2. What are the diagnostic points in favor of SCC?
• Ulceroproliferative lesion
• The ulcer is having everted edges of SCC
• The proliferative area has cauliflower-like
appearance
• The copious bloody foul smelling discharge
• Induration of the base
• Enlarged regional lymph nodes.
Q 3. What is the origin of SCC?
• Itis a malignant tumor of the keratinizing cells
of the epidermis or its appendages
• It can also arise from the stratum basale of the
epidermis
• It expresses cytokeratin 1 and 10.
Q 4. Are these nodes really metastatic?
The lymph nodes are not very hard in this case.
When the lymph nodes are palpable, in about 1/3rd
of the patients the adenopathy may be secondary
to infection, that will subside after treatment of the
primary lesion.Until proved otherwise, itshould be
assumed that they are metastatic.
Q 5. What are the differential diagnoses of a small
lesion?
• Solar keratosis (Actinic keratosis)
• Basal cell carcinoma
• Keratoacanthoma
• Pyogenic granuloma
• Seborrheic warts.
Q 6. What are the premalignant (precancerous)
conditions of the skin?
Precancerous lesions of the skin
• Senile keratosis
• Arsenic dermatitis
• Leukoplakia
• Solar keratosis (Actinic keratosis)
• Radiation dermatitis
• Kraurosis vulvae
• Xeroderma pigmentosum
• Junctional nevus
• Albinism
• Cutaneous horn
• Keratoacanthoma—Papilloma virus infection,
smoking are associated—seen in old age
• Bowens disease—this is SCC in situ (HPV 16 is
implicated)
• Extramammary Paget’s disease—intraepidermal
adenocarcinoma
• GCPN (Giant Congenital Pigmented Nevus)—for
malignant melanoma.
Q 7. How about Paget’s disease, Bowen’s disease
and Erythroplasia of Queyrat?
Paget’s disease, Bowen’s disease and Erythroplasia of
Queyrat are carcinoma in situ (Paget’s disease is seen
in nipple, erythroplasia of Queyrat is seen in penis).
Squamous Cell Carcinoma—SCC (Epithelioma)
409
Q 14. What are the mechanisms by which the UV
radiation affects the skin? (PG)
It affects the skin in two ways:
1. Direct carcinogenic effect on dividing keratinocytes in the basal layer of the epidermis.
2. Depression of the cutaneous immune surveillance response.
Q 15. What are the sites of SCC other than skin?
The SCCisseeninthe followingsitesotherthanskin.
• Lips
• Mouth
• Pharynx
• Esophagus
• Anal canal
• Glans penis
• Uterine cervix
• Metaplastic areas of respiratory epithelium.
Q 16. What are the other predisposing factors?
Predisposing factors for SCC
1. Sunlight
2. Susceptible phenotype
3. Compromised immunity—(after 10 years of
immunosuppression, 10% will develop malignancies—may be secondary to HPV virus)
4. Chemicals—For example, Hydrocarbons (soot),
arsenical, tar, etc.
5. Infection—HPV 5 and HPV 16
6. Mineral oils
7. Arsenic
8. Chrome compounds
9. Chronic inflammation—Chronic sinus tract,
pre-existing scars(Marjolin’s ulcer), osteomyelitis,
burns and vaccination points
10. Immunosuppression (organ transplantrecipients)
11. Smoking—current and previous tobacco use.
Q 8. What is the difference between precancerous
lesion and carcinoma in situ?
The precancerous lesions will lead on to in situ
cancer and finally cancer.
Precancerous lesion
↓
In situ carcinoma (preinvasive)
↓
Carcinoma
Q 9. What are the features of solar keratosis
(Actinic keratosis)? (PG)
They are discrete, scaly, irregular patches, which
may project occasionally.
Q 10. What is the treatment of actinic keratoses?
(PG)
They are treated with:
• Cryotherapy
• Topical 5 Fu—(5 fluorouracil)
• Electrodesiccation and curettage
• CO2 Laser
• Dermabrasion.
Q 11. What are the features of Bowen’s disease?
(PG)
They are irregularly shaped, well-defined plaques
resembling a patch of eczema having beefy red,
erythematous raised scaly crust on the surface.
Q 12. What are the features of erythroplasia? (PG)
They are having the same features as Bowen’s
disease, except for the fact that they are situated
on the penis.
Q 13. Which part of the ultraviolet ray is responsible
for skin damage?
Ultraviolet B is thought to be the form of radiation
responsible for the damage by sunlight.
410
Clinical Surgery Pearls
Q 17. What is “Chimney sweeps” cancer?
The soot is one of the most important hydrocarbons
responsible for squamous cell carcinoma of the
scrotum in chimney sweeps. Itis also seen inpeople
who work in tar.
Q 18. What is “Khangri” cancer?
Khangriisanearthenware,filledwithburningcharcoal.
The Kashmiris keep the Khangri on their abdomen
to keep themselves warm. The patient will develop
squamous cell carcinoma of the abdominal wall.
Q 19. What is Kang cancer?
This is a squamous cell carcinoma developing in
the buttocks, back, heels and elbows. This is seen
in Tibetans who sleep on the oven bed.
Q 20. What is countryman’s lip?
This is nothing but SCC of the lower lip seen in
farmers as a result of sun exposure.
Q 21. What are the macroscopic types of SCC?
Three types are seen:
1. Ulcerative
2. Proliferative (Cauliflower-like)
3. Ulceroproliferative.
Q 22. What is the microscopic pathology of SCC?
• Epithelial pearls (Cell nests)—Squamous cells
arrangedinconcentricmannersuchasonionskin
• Plasma cell infiltration
• Positive for cytokeratins 1 and 10.
Q 23. What is the spread of SCC?
• Local spread—to the subcutaneous tissue,
tendons, muscles, bone and vasculature
(Involvement ofthe local blood vessel can cause
thrombosis and ischemia. The subcutaneous
spread may involve the nearby nerves causing
neuritis—perineural involvement)
• Themainspreadislymphatic reachingthe lymph
node
• Bloodstream spread occurs very rarely—(to the
lungs).
Q 24. Can you get SCC without lymph node
metastasis?
Yes. Lymph node spread is absent in the following
situations.
• Whenthe lesionisdevelopinginscar andchronic
ulcer (Marjolin’s ulcer)
• SCC in old age.
Q 25. What are the characteristics of Marjolin’s ulcer?
Characteristics of Marjolin’s ulcer
• It arises from ulcer or scar
• The edge is not always raised and everted
• It is not very invasive
• The growth is very slow
• More aggressive than spontaneous SCC.
Q 26. What are the aggressive types of squamous
cell carcinoma? (PG)
1. Squamous cell carcinomas seen in transplant
patients
2. Squamous cell carcinomas developing in scars
and ulcers
3. Anaplastic squamous cell carcinoma.
Note: Metastasis is more likely to arise from SCC in
scars and ulcers even though the lymph nodes are
not involved.
Q 27. What are the types of lesions prone for
malignant change resulting in Marjolin’s ulcer?
Lesions prone for Marjolin’s
1. Venous ulcers
2. Chronic ulcers
3. Scar tissues—Postburn scarring
4. Scarring secondary to lupus vulgaris (tuberculosis)
5. Chronic discharging sinuses, e.g. osteomyelitis.
Squamous Cell Carcinoma—SCC (Epithelioma)
411
Q 28. How aggressive is SCC?
The SCC is more aggressive than BCC and therefore
wider excision margins are required for local control.
Q 29. What is the type of biopsy recommended
for SCC?
• Incision biopsy from the edge of the ulcer if the
lesion is large (wedge biopsy from the edge)
• If the lesion is small excision is recommended.
Q 30. Why the edge of the ulcer is preferred for
incision biopsy?
The edge of ulcer is the growing part which will
show the malignant cells.
Q 31. What are the other investigations required?
• X-ray of the affected part to rule out bone
involvement
• X-ray of the chest to rule out metastasis (very
rare event)
• Other investigations required for anesthesia
clearance.
Q 32. What is the staging of SCC? (PG)
The TNM staging is used (this is different from the
TNM of the melanoma).
Primary tumor (T)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 Tumor 2 cm or less in greatest dimension with
less than 2, high-risk factors
T2 Tumor greater than 2 cm, or tumor of any size
with 2 or more high risk features
T3 Tumor with invasion of maxilla, mandible,
orbit or temporal bone
T4 Tumor invasion of skeleton (axial or
appendicular) or perineural invasion of skull
base
Note: In case of multiple simultaneous tumors, the
tumor with highest T category will be classified.
Regional lymph node (N)
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1, N2a, N2b, N2c & N3 as in head and neck
malignancy (Read case no: 24)
Distant metastasis (M)
MX Distant metastasis cannot be assessed
M0 No distant metastasis
M1 Distant metastasis
Stage grouping
Stage 0 Tis N0 M0
Stage 1 T1 N0 M0
Stage II T2 N0 M0
Stage III T3 N0 M0
T1 N1 M0
T2 N1 M0
T3 N1 M0
Stage IV T1 N2 M0
T2 N2 M0
T3 N2 M0
Any T N3 M0
T4 Any N M0
Any T Any N M1
Q 33. What is the importance of grade in SCC?
Grade has been included as one of the high-risk
features within the T category and now contributes
towards the final stage grouping. The grades are:
Gx – Grade cannot be assessed
G1 – Well differentiated
G2 – Moderately differentiated
G3 – Poorly differentiated
G4 – Undifferentiated
Q 34. What are the high risk features of SCC?
1. Histologic grade
2. Primary anatomic site—ear or hair bearing lip
3. More than 2 mm depth
4. Clark level more than IV
5. Perineural invasion.
412
Clinical Surgery Pearls
Q 35. What are the bad prognostic factors for
SCC? (PG)
Tumors of the following regions are having bad
prognosis:
1. Site of the tumor: Ears and lips are having bad
prognosis. Tumors of the extremities fare worse
than the trunk.
2a.Depth of invasion: Less than 2 mm metastasis
unlikely
More than 6 mm—15% will have metastasis
2b.Surface size: Lesion more than 2 cm have worse
prognosis than smaller ones
3. Histological grade:Higherthe Broder’s grade the
worse the prognosis
4. Etiology: SCC from burn scars, osteomyelitis,
sinuses, chronic ulcers and areas that have been
irradiated have a higher metastatic potential
5. Immunosuppression: Bad prognosis
6. Perineural involvement: Worse prognosis and
require wider excision.
Q 36. What is the histological grading of SCC? (PG)
GX – Grade cannot be assessed
G1 – Well-differentiated
G2 – Moderately differentiated
G3 – Poorly differentiated
G4 – Undifferentiated
Q 37. What is the staging in this case? (PG)
It is stage II or III. (If the lymph node is positive).
Q 38. What are the options for treatment?
The treatment options are:
1. Surgical excision
2. Destructive therapy
3. Radiotherapy.
Q 39. What is the recommended excision margin
for SCC?
• For lesions less than 2 cm diameter—4 mm
margin is adequate
• For more than 2 cm lesions—1 cm margin is
recommended.
Q 40. What would be the treatment of choice in
this case?
• Wide excision with 1 cm clearance
• Split skin grafting of the raw area.
Q 41. What are the areas where a skin graft will
not take? (PG)
The skingraftwillnottake inthe followingsituations:
• Exposed cortical bone without periostium
• Cartilage without perichondrium
• Tendon without paratenon
• Irradiated tissue.
Q 42. What sort of cover is recommended in such
situations?
A flap is recommended.
Q 43. What are the indications of the flap? (PG)
Flaps are indicated in the following situations:
1. Areas where graft will not take
2. Where there isrisk ofscar contracture especially
across a joint
3. Where esthetic result is important
4. Where bulk or structural support is needed.
Q 44. What are the types of flaps? (PG)
It may be Local flap
Island flap
Free flap
Q 45. How do you manage the enlarged inguinal
nodes?
• Give a course of antibiotics and see whetherthe
nodes are subsiding or not
• If the nodes are remaining the same after 3
weeks, FNAC of the node is done
• Ifthe FNAC ofthe node is positive for metastasis,
an inguinal block dissection is carried out.
Squamous Cell Carcinoma—SCC (Epithelioma)
413
Q 46. If the lesion is involving the bone, what
would be the treatment option? (In given case)
The primary needs below knee amputation and the
inguinal nodes are managed as mentioned above.
Q 47. What are the indications for radiotherapy?
Radiotherapy has been shown to cure 90% cases
of SCC:
It is recommended for the following situations:
• Debilitated patients
• Poor surgical risk candidates
• Those who refuse surgery.
Q 48. What are the problems of radiotherapy? (PG)
• Unpleasant side effects
• Protracted treatment.
Q 49. Is there any role for radiotherapy as an
adjuvant treatment? (PG)
Yes. The indications are:
• High stage large tumors
• Recurrent tumors.
Q 50. Is there any role for topical 5–fluorouracil
(5-FU)? (PG)
No, it is not recommended for the primary
treatment of SCC.5-FU is an excellent method of
treating, premalignant lesions associated with SCC
such as actinic keratoses.
Q 51. What is the role of Mohs micrographic
technique in SCC? (PG)
This technique is also used for the treatment of SCC.
The advantages are:
• Tissue preservation
• Lower recurrence rate.
The disadvantages are:
• Patient inconvenience
• Expensive.
Q 52. What is the role of destructive techniques in
the treatments of SCC (Cryosurgery and electrodesiccation and curettage)? (PG)
• They are best reserved for very smallsuperficial
lesions in noncritical areas
• The local failure rate is high
• They do not produce surgical specimen for
histological examination and margin analysis
• The healing is by secondary intention
• It results in poor scars.
Q 53. Is there any role for prophylactic lymph node
dissection? (PG)
No.
40 Carcinoma Penis
Case
Case Capsule
A 50-year-old Hindu male patient presents
with acquired phimosis of recent onset with
seropurulent discharge from beneath the prepuce.
On examination, the penis is swollen at its tip. There is
a hard mass felt beneath the prepuce on palpation.
On forceful retraction, a part of the ulceroproliferative
lesion is seen protruding from the glans. Palpation of
the corpora cavernosa appear normal. There is no
induration. On examination of the inguinal nodes,
two horizontal group of inguinal nodes which are
firm in consistency and mobile are felt on either side.
There are no enlarged iliac nodes. The rest of the
external genitalia and abdomen are normal.
Read the checklist for examination of the ulcer.
Checklist for examination
• Assess the location of the lesion
• Assess the size
• Decide whether it is mobile or fixed
• Palpate the penis for involvement of the corpora
(induration)
• Check for involvement of surrounding structures
like scrotum and perineum
• Rectal examination for involvement of perineal
body and pelvic lymph nodes
• Examination ofinguinal area foringuinal nodes and
iliac nodes
• Remember the three most important examinations
in the male genitalia
• Always retract the prepuce and see for any lesion
• Always examine the under surface of the penis for
openings (hypospadias)
• Always examine the ventralsurface of the scrotum
for openings (hypospadias).
Contd...
Contd...
Carcinoma Penis
Q 1. What is the most probable diagnosis in this 415
case?
Carcinoma penis with acquired phimosis and?
Inguinal node metastasis.
Q 2. What are the differential diagnoses?
• Syphilitic chancre
• Soft chancre due to Haemophilus ducreyi
• Giant condyloma.
Q 3. What is phimosis?
Inability to retract the foreskin to expose the glans.
Q 4. What is paraphimosis?
Inability to reduce a previously retracted foreskin.
Q 5. What is the importance of religion in this case?
Hindus are usually not circumcised and therefore
more prone for carcinoma of the penis.
Q 6. What is the commonest age group affected?
• 40%ofthepatients are underthe age of 40 years.
• It is commonly seen in middle and old age.
Q 7. What is balanitis?
Balanitis is an infection of the glans penis.
Q 8. What is balanoposthitis?
It is commonly used to describe an infection within
the preputial sac which, affects both the surfaces of
the glans penis and the inner aspect of the prepuce.
416
Clinical Surgery Pearls
Q 16. What are the sites where you can get
leukoplakia?
• Oral cavity
• Tongue
• Vulva
• Vagina
• Penis.
Q 17. What is the appearance of erythroplasia of
Queyrat?
It presents as a flat dark red slightly indurated patch.
Q 18. What is Balanitis xerotica obliterance (lichen
sclerosus atrophicus)?
It is a condition with unknown etiology. The lesion
appears as white plaques on the surface of glans
and prepuce. The foreskin is thickened, fibrous
and difficult to retract. It is seen in men aged 20-40
years and they have higher incidence of associated
autoimmune disorders. Circumcision is indicated
in this condition. It may also cause meatal stenosis.
This is not a premalignant lesion.
Q 19. What are the causes for acquired phimosis?
• Cancer
• Chancre
• Balanoposthitis.
Q 20. What is the management of acquired
phimosis?
If retraction of the prepuce is not possible arrange
for a dorsal slit of the prepuce and examine the
inside or arrange for a circumcision.
Q 21. What are the macroscopic types of carcinoma
penis?
• Flat type (infiltrative type)
• Papillary type with wide sessile pedicle
• Ulcerative type.
Q 9. What are the causes for balanoposthitis?
Causes for Balanoposthitis
1. Carcinoma of penis
2. Nonspecific infection secondary to poor hygiene
3. Phimosis in diabetic patients
4. Primary chancre.
Q 10. What is the most important causative factor
for carcinoma penis?
Human papilloma virus 16 (HPV 16).
Q 11. What are the other predisposing factors?
• Smoking
• Smegma
• Poor hygiene
• Chronic balanoposthitis.
Q 12. Will circumcision confer immunity against
carcinoma penis?
• Circumcision soon after birth confers complete
immunity against carcinoma
• Later circumcision does not have the same
effect.
Q 13. What are the premalignant conditions?
• Leukoplakia of the glans (similar to the lesion
seen in the tongue)
• Longstanding genital warts(chronic papilloma)
• Chronic balanitis.
Q 14. What is erythroplasia of Queyrat and Paget’s
disease?
They are nothing but in situ carcinoma presenting
as persistent rawness of the glans.
Q 15. What is the appearance of leukoplakia?
It is identical to the appearance of leukoplakia of the
tongue presenting as patches of grayish white paint.
Carcinoma Penis
417
Q 22. What are the modes of spread of carcinoma
penis?
1. Local spread
2. Lymphatic spread
3. Bloodstream spread (distant metastasis).
Q 23. What is the barrier for the local spread?
The fascial sheath of the corpora cavernosa.
Q 24. What is lymphatic drainage of penis?
• The superficial lymphatics drain to the inguinal
nodes
• The deep lymphatics drain to the iliac nodes.
Q 25. What is the mode of transmission of HPV
virus?
The HPV infection directly correlates with the
number of life term partners (however carcinoma
penis cannot be labelled as a sexually transmitted
disease.
Q 26 . What are the locations for SCC of the penis?
• Glans (50%)
• Prepuce
• Coronal sulcus.
Q 27. What is Buschke-Loewenstein tumor?
Buschke-Loewenstein tumor has the following
characteristics:
• It has got histological pattern of verrucous
carcinoma
• It is locally destructive and invasive
• No nodal metastasis is seen
• No distant metastasis
• Treatment is surgical excision.
Q 28. How do you confirm the diagnosis?
• Incision biopsy from the lesion (if it is ulcerative
take the biopsy from the edge)
• If acquired phimosis is there—do a dorsal slit
of the prepuce and do incision biopsy from the
lesion
• Excision biopsy for very small superficial lesions
of the glans
• Circumcision and biopsy if the lesion is confined
to the prepuce.
Q 29. What is the commonest pathological type?
Squamous cell carcinoma.
Q 30. Can you get metastasis in penis?
Yes. The usual sites for primary are urinary bladder,
prostate and rectum.
Q 31. What are the other investigations required?
1. Ultrasound of the penis
• To evaluate the depth of invasion
• Detecting involvement of the corpus
cavernosum.
2. Contrast enhanced MRI—for lesions suspected
to invade corpora
3. CT—for evaluation of the inguinal and pelvic
nodes (physical examination is enough in the
ordinary circumstances)
4. X-ray chest.
Q 32. What is the staging of carcinoma penis? (PG)
The AJCC 7th edition TNM classification is used.
Summary of changes
1. T1 has been subdivided into T1a and T1b based
on the presence or absence of lymph vascular
invasion (LVI) or poorly differentiated cancer.
2. T3 is limited to urethral invasion.
3. Prostatic invasion is now considered T4.
4. Nodal staging is divided into both clinical and
pathologic categories.
5. The distinction between superficial and deep
inguinal nodes has been eliminated.
6. Stage II grouping includes T1b N0 M0 as well as
T2–3 N0 M0
• TX – Primary Tumor cannot be assessed
• T0 – No evidence of primary tumors
418
Clinical Surgery Pearls
• Tis – Carcinoma in situ
• Ta – Noninvasive verrucous carcinoma
• T1a – Tumorinvadessubepithelial connective tissue without lymph vascular
invasion and is not poorly differentiated
• T1b – Tumor invades subepithelial connective tissue with lymph vascular
invasion or is poorly differentiated
• T2 – Tumorinvades corpusspongiosumor
cavernosum
• T3 – Tumor invades urethra
• T4 – Tumorinvadesotheradjacentstructures
Regional Lymph Nodes (N)
• cNX – Regional lymph nodes cannot be
assessed
• cN0 – No palpable or visibly enlarged
inguinal lymph nodes
• cN1 – Palpable mobile unilateral inguinal
lymph node
• cN2 – Palpable mobile multiple or bilateral
inguinal lymph nodes
• cN3 – Palpable fixed inguinal nodal mass or
pelvic lymphadenopathy unilateral or
bilateral.
Note: Clinical stage definition based on palpation,
imaging
Pathologic stage definition (based on biopsy or
surgical excision)
pNX – Regionallymphnodes cannotbeassessed.
pN0 – No regional lymph node metastasis.
pN1 – Metastasisina single inguinal lymph node
pN2 – Metastasisin multiple or bilateral inguinal
lymph nodes
pN3 – Extra-nodal extension of lymph node
metastasis or pelvic lymph nodes(s)
unilateral or bilateral.
Distant Metastasis (M)—lymph node metastasis
outside the true pelvis in addition to visceral or
bone sites
• MX – Distant metastasis cannot be assessed
• M0 – No distant metastasis
• M1 – Distant metastasis
Stage Grouping
Stage 0 Tis N0 M0
Ta N0 M0
Stage I T1a N0 M0
Stage II T1b N0 M0
T2 N0 M0
T3 N0 M0
Stage IIIa T1-3 N1 M0
Stage IIIb T1-3 N2 M0
Stage IV T4 Any N M0
Any T N3 M0
Any T Any N M1
Q 33. What is the most important prognostic
factor for survival in carcinoma penis?
Presence of metastasis to the inguinal nodes.
Q 34. What are the predictors for nodal involvement? (PG)
Predictors for nodal involvement
• Grade of the tumor (higher the grade more chance
for involvement of nodes)
• Corporal involvement
• Vascular involvement
• Lymphatic embolization.
Carcinoma Penis
419
Q 35. What are the treatment options?
Treatment options for carcinoma penis
• Surgery (amputation of penis—partial or total)
• Primary radiation therapy for the primary
• Laser therapy—CO2 laser/Nd: YAG laser (for very
small non infiltrating lesions)
• 5-FU cream (for early lesions)
• Treatment ofinguinal nodes—unilateral or bilateral
lymph node dissection (inguinal) or bilateral
inguinal irradiation
• Adjuvant chemotherapy
• Reconstruction of the penis if suitable.
Note: Stage 1 and 2 lesions can be treated with
radiotherapy with a good cosmetic and functional
result.
Q 36. What are the indications for radiation
therapy for the primary?
Indications for radiotherapy
• Young patients with small lesions (2–4 cm)
• Superficial lesions
• Exophytic lesions
• Noninvasive lesions on glans or coronal sulcus
• Patients refusing surgery
• Patients with inoperable tumors.
Q 37. What are the types of radiotherapy?
• External beam radiation
• Interstitial brachytherapy—Iridium 192 or
Tantalum wire or Cesium 137
• Radioactive mould application (applied externally to the penis).
Q 38. What are the indications for organ
preservation?
• Tis,Ta andT1 Infiltration ofthe shaft ofthe penis
• Large anaplastic growth
• Failure of radiotherapy.
Q 39. What are the indications for partial amputation of penis?
• T2 to T4-tumors
• If preservation of 2 cm penile stump is possible
after 2 cm clearance from the gross tumor.
Q 40. What are the advantages of partial amputation of penis?
• Less psychological trauma
• Ability to pass urine in standing position
• Preservation of sexual function.
Q 41. What is the minimum clearance required in
carcinoma penis?
Well and moderately differentiated tumors need
only 1cm margin.
Q 42. What is glansectomy? (PG)
• This is reserved for verrucous carcinoma and
minimally invading T1 lesion
• It preserves more erectile tissue.
Q 43. What are the features of verrucous carcinoma
and basaloid carcinoma?
Verrucous carcinoma Basaloid carcinoma
They are well differentiated Poorly differentiated
Presence of expansile
border
Infiltrative
Non-metastatic Frequently metastatic
Q 44. What is total amputation of penis?
• Thisis done for advanced lesions, and anaplastic
lesions
• Perineal urethrostomy is done aftertotal amputation.
Q 45. Is there any need for bilateral orchidectomy
along with total amputation of the penis?
• The traditional arguments in favor of bilateral
orchidectomy will not hold today (soiling of
420
Clinical Surgery Pearls
scrotum during urination and edema scrotum
after inguinal block dissection)
• Thepatientwill loosehishormone (testosterone)
and masculine features by doing orchidectomy
• Therefore, orchidectomy is not recommended.
Q 46. What are the indications for inguinal
lymphadenectomy (inguinal block dissection)?
There are five groups of inguinal lymph nodes:
Central, Superolateral, Inferolateral, Superomedial
and Inferomedial. The superomedial is called
Cabana’s node. The indications for inguinal
lymphadenectomy are:
• The nodes are usually managed after controlling
the primary tumor and a course of antibiotics
• In palpable adenopathy, there is a higher
likelihood of finding metastasis and a lower
survival and therefore lymphadenectomy is
justified
• T2 to T3 tumors without palpable inguinal
adenopathy
• Tumors exhibiting lympho vascular invasion
• Poorly differentiated tumors even without
invasion of corpora cavernosum or spongiosum.
Q 47. What are the situations where lymphadenectomy is not required? (PG)
• PatientswithTis,TaandT1tumorswithoutlympho
vascular invasion and without poor differentiation
and absence of palpable adenopathy.
Q 48. What is Cabana’s node? (PG)
• Cabanadescribedaprocedureof sentinel lymph
node biopsy for metastasis from carcinoma penis
• These nodes are situated superomedial to the
junction of the long saphenous vein with femoral
vein in the area of superficial epigastric vein
• Ifthe sentinelnodes arenegative formalignancy,
thenthereisnoneedforinguinalblockdissection
• The technique involves peri-tumoral injection
of Technetium 99m and blue dye
• It is not being used widely
Q 49. What is the lymphatic drainage of the
corpora? (PG)
The corpora will drain directly to the deep inguinal
nodes (Rosenmüller’s or Cloquet’s node).
Q 50. What is the reason for recommending
bilateral lymph node dissection when the nodes
are positive? (PG)
It is because of the anatomic cross over the penile
lymphatic.
Q 51. What type of inguinal dissection is recommended? (PG)
Initially a superficial inguinal dissection is recommended, which involves removal of the nodes
superficial to fascia lata.These nodes are subjected
for frozen section and if found to be positive the
patient is subjected for complete ilioinguinal and
pelvic lymph node dissection.
Q 52. What are the complications of inguinal block
dissection?
Complications of inguinal block dissection
• Wound infection
• Flap necrosis
• Lymph edema of the lower limb
• Lymph edema of the scrotum.
Q 53. What is the indication for bilateral inguinal
irradiation? (PG)
• This is usually done for N0 nodes
• Not recommended for high-risk patients
• Maybehelpful infixed ulcerated inguinal nodes
as palliative procedure
• Preoperative radiationfordown staginginguinal
nodes.
Carcinoma Penis
421
Q 54. Is there any role for adjuvant chemotherapy? (PG)
Yes
• If more than two histological positive nodes
• If extra-nodal extension of cancer is present.
Q 55. What are the chemotherapeutic agents
used? (PG)
• Cisplatinum based regimens are used (5-FU,
bleomycin and methotrexate)
• Ifosfamide (new drug).
Q 56. Is there any role for penile reconstruction? (PG)
Reconstructions are being tried after total
amputation of penis with variousflaps. Restoration
of phallus with tactile and erogenous sensation,
creation of urethra and enough bulk are important
for a successful reconstruction.
Q 57. What is the management of distant
metastasis? (PG)
Chemotherapy with cisplatin and methotrexate.
Q 58. What is the cause of death in carcinoma
penis?
It is usually by the metastatic nodes eroding into
the femoral or external iliac artery with torrential
hemorrhage
Q 59. What is the prognosis of carcinoma penis?
• The five yearsurvival for lesionslocalized to the
penis is 80%
• With nodal metastasis the five year survival is
50%
• With distant metastasis the five year survival is
nil.
41 Congenital Arteriovenous Fistula/
Hemangioma/Compressible Swelling
Case
Case Capsule
A 25-year-old male presents with dilated tortuous
pulsatile vessels on the entire left lower limb with
increased length and girth of the limb suggestive
of local gigantism. There is scoliosis of the spine.
On examination there is port wine discoloration of
the lateral part of the left thigh. Palpation revealed
pulsations of the tortuous vessel and these vessels
were compressible and clinically appearing to be
veins. Palpation revealed continuous thrill over
the vessels. The extremity is appreciably warmer
and moist than the unaffected side. Auscultation
revealed a continuous machinery murmur with
systolic accentuation. Examination of the radial pulse
revealed collapsing radial pulse. After occlusion of
femoral artery, the bradycardiac sign was positive.
There was no evidence of cardiac failure.
Read the checklist for examination of the swelling.
Checklist for history
• Find out whether the lesion is present from birth
or not
• Find out whether there is rapid postnatal growth
and slow involution (hemangioma)
• Findoutwhetherthelesionishavingcommensurate
growth as the age advances
• Historyofhemorrhage (GI bleed) and local bleeding
• History of ulceration of the limb or lesion
• History of stridor (subglottic hemangioma)
• History of sudden increase in size (bacterial
infection, or secondary to hormonal changes).
Checklist for examination
• Examine the radial pulse and decide whether it is a
collapsing pulse or not
• Look for skin discoloration
• Look for compressibility and decide whether it is
partially compressible or completely compressible
• Check whether the swelling enlarges with
dependency and disappears with elevation of
the involved limb
• Palpate for increased local warmth
• Rule out increased moisture compared to the
normal limb
• Check whether the overlying skin is normal or not
(dystrophic changes in the skin)
• Look for discrepancy in the limb length and if it is
there apparently, always take measurements
• Look forincrease in girth by taking measurements
in all segments of limb
• Lookforatrophyoftheaffectedarea(bone atrophy as
a result of reduction in distal circulation and hypoxia)
Contd...
Contd...
Contd...
Congenital Arteriovenous Fistula/Hemangioma/Compressible Swelling
423
• Look for features of platelet trapping
• Look for translucency
• Look for palpable thrill
• Look for continuous bruit
• Look forbradycardiac sign after occluding the main
feeding artery
• Always examine the heart especially for evidence
of cardiac failure
• Look for hepatomegaly.
Contd...
424
Clinical Surgery Pearls
Q 5. What is the new classification?
The new classification distinguishes lesions as
follows:
• Lesionsthat regressspontaneously— Hemangiomas
• Those that do not regress spontaneously—
Congenital vascular malformations (Hamburg
classification—Predominantly arterial, venous,
lymphatic, arteriovenous shunting and mixed).
Another classification is
• Slow flow lesions
• Fast flow lesions—Arteriovenous fistula.
Q 6. Which channel is more affected in congenital
vascular malformations?
• Venous defects are the most common
• Arteriovenous malformations makeup 1/3rd of
the lesions
• About 90% of the arteriovenous malformations
occur in the extremities, pelvis, trunk and
shoulder girdle.
Q 7. What are the differences between hemangioma and vascular malformation?
• Hemangiomas result from cellular proliferation
(It is a fibrofatty structure and the contained
blood cannot be evacuated completely). The
hemangiomas grow over the first six to eight
months of life. After 1 year signs of involution
appear until about 5–10 years old.
• Vascular malformations are embryonic and
developmental abnormalities (error in vascular
morphogenesis). These lesions grow parallel
with the age, they may expand suddenly at times
with associated infections. There is no involution.
Limb hypertrophy or atrophy may occur.
Q 1. What are the differential diagnoses for a
compressible swelling?
1. Lymphatic cyst/lymphangioma
2. Hemangioma
3. Aneurysm
4. Arteriovenous fistula.
Q 2. What are the clinical points to differentiate
these four conditions?
• The lymph cyst will be brilliantly transilluminant
• Hemangioma is nottransilluminant,butpartially
compressible
• Aneurysm will show expansile pulsations and
systolic bruit
• Arteriovenous fistula will show continuous
thrill on palpation and continuous machinery
murmur on auscultation.
Q 3. What is the difference between cavernous
hemangioma and capillary hemangioma?
These are old terms and they are best avoided.
Cavernous hemangioma is used to describe a
deep lesion involving the deeper dermis or the
subcutaneous tissue and capillary hemangioma
is one which proliferates in the superficial
dermis.
Q 4. What is the old classification of hemangioma?
They are classified into capillary and cavernous
(not used now).
• Capillary hemangioma – Strawberry
– Port wine stain
– Spider nevi
• Cavernous hemangioma
Note: The term cavernous hemangioma is not there
in the current terminology. Most of the lesions are
actually venous malformations.
Congenital Arteriovenous Fistula/Hemangioma/Compressible Swelling
425
Differences between hemangioma and vascular
malformations
Hemangioma Vascular malformation
• Usually present at birth • Present at birth, may
not be apparent
• Result from cellular
proliferation (Benign
tumor)
• Embryonic and
developmental
abnormalities
• Fibrofatty structure
and contained blood
cannot be evacuated
completely by
compression
• Easily evacuated by
compression
• No enlargement with
dependency and
disappearance with
elevation of the limb
• Enlarges with
dependency and
disappears with
elevation of the limb
• Endothelial hyperplasia • Flat endothelium
• Female to male ratio 5:1 • Female to male ratio 1:1
• Mast cells are increased • Normal mast cells
• Multilaminated
basement membrane
• Unilaminated basement
membrane
• Platelet trapping
present (Kasabach
Merritt’s syndrome)
• No platelet trapping
• Rapid postnatal growth
with slow involution
• Commensurate growth
No spontaneous
involution
• No limb hypertrophy/
atrophy
• Limb hypertrophy or
atrophy may occur
• No treatment required
in majority
• Treatment may be
required
Q 8. What are the examples of vascular malformations?
• Port wine stain (capillary malformation)
• Nevi
• Venous malformations
• Lymphatic malformation
• Arteriovenous fistula.
Q 9. What is the classical appearance of a
strawberry hemangioma?
It looks like a raised, bright red patch with a
textured surface like a strawberry. Veins radiating
from the tumor may be seen beneath the skin.
During proliferative phase the tumor enlarges and
becomes brighter in color. Involution is heralded by
softening and fading of color.
Q 10. What are the classical sites of hemangioma?
Sites of hemangioma
• Head and neck (60%)
– Facial hemangioma
– Eyelid hemangioma can cause astigmatism and
amblyopia
– Subglottic hemangioma causing biphasic stridor
• Trunk (25%)
• Limbs (15%)
• Liver
– Multiple intrahepatic hemangiomas
– Hepatomegaly
– Heart failure
– Anemia
• Intestines
• Lungs
• Brain.
Note: 80% are single tumors and 20% are multiple
tumors.
426
Clinical Surgery Pearls
Q 11. What are the complications of hemangiomas?
Complications of hemangiomas
• Platelet trapping
– Kasabach—Merritt syndrome
• Thrombocytopenia and hemorrhage
– Gastrointestinal
– Pleural
– Intracranial
– Intraperitoneal
• Ulceration (5%)
• Local bleeding
• Infection
• Visual problems by obstructing the vision
• Stridor.
Q 12. What is the Kasabach-Merritt syndrome?
Thiswas described by Kasabach and Merrittin 1940.
It is characterized by:
Kasabach-Merritt Syndrome
• Hemangioma > 5 cm
• Thrombocytopenia
• Bleeding diathesis.
The thrombocytopenia is by platelet trapping.
There is 37% mortality for this condition which is
largely due to bleeding. The treatment is difficult
and interferon, steroids and irradiation have all been
tried with variable results.
Q 13. What is Hamburg classification?
Humburg classification isfor vascular malformation
based on the predominant nature of the
malformation. They are classified into five types—
predominantly arterial defects, predominantly
venous defects, predominantly lymphatic defects,
predominantly AV shunting defects and combined
or mixed vascular defects.
Hamburg classification of congenital vascular defects
Type
Forms
Truncular Extra truncular
Predominantly arterial defects Aplasia or Obstructive dilation Infiltrating or limited
Predominantly venous defects Aplasia or Obstructive dilation Infiltrating or limited
Predominantly lymphatic defects Aplasia or Obstructive Infiltrating or limited
Predominantly AV shunting defects Deep or superficial Infiltrating or limited
Combined/mixed vascular defects Arterial and venous, no AV shunt
Hemolymphatic, with or without
AV shunt
Infiltrating hemolymphatic or limited
hemolymphatic
Congenital Arteriovenous Fistula/Hemangioma/Compressible Swelling
427
Other classifications are:
A. Schobinger classification: Quiescent,
Expanding
Destruction
Decompensation
B. International society on studies of vascular
anomalies (ISSVA)
Slow flow, Fast flow & Complex
Class I – Vascular tumors
- Infantile hemangioma
- Congenital hemangioma
Rapidly Involuting
Congenital Hemangioma
(RICH) andNoninvoluting
congenital hemangioma
(NICH)
- Kaposiform hemangioen-
dothelioma
- Dermatologically acquired
vascular tumor like pyo- genic granuloma
Class II – Vascular malformation
a. Slow flow—Capillary
hemangioma and port
wine stain
b. Fast flow—Arterial fistula,
AV malformation and AV
fistula
c. Complex
C. Mulligan classification
– Truncal, diffuse and localized
Q 14. What are the associations of congenital
vascular malformations?
• Nevi
• Port wine stain
• Varicosities
• Arteriovenous fistula
• Hypertrophy and atrophy of the extremities
• Edema of the limbs.
Q 15. What is port wine stain (in old classification
it was included with hemangioma)?
This is an extensive intradermal capillary
malformations giving a deep purple color to the
overlying skin.They are present at birth, commonly
seen on the face, at the junction between limbs and
trunk (shoulders, neck and buttocks). Sometimes
they are seen distributed along sensory branches
of the fifth cranial nerve. Microscopically they are
formed by thin-walled capillaries in the dermis.
They are non-involuting lesions like any other
arteriovenous malformation (unlike hemangiomas).
Q 16. What are the treatment options of port
wine stain?
• Camouflaging andTattooing are the optionsfor
the management
• Flash lamp pulsed—dye laser(multiple sessions
are necessary)
• Selective photo thermolysis
• If laseris unsuccessful,surgical excision and skin
grafting.
Q 17. What is the age group for lymphatic
malformations and what is the presentation?
They are seen in the first year of life and childhood.
They present as lymphatic vesicles.
Q 18. What is lymphangioma/cystic hygroma?
It is a localized cluster of dilated lymph sac in
the skin and subcutaneous tissues which do not
connect into the normal lymph system (they are
clusters of lymph sacs that fail to join into the
lymphatic system during development). When they
are large, cystic and translucent and confined to the
subcutaneous tissue, they are called cystic hygroma.
428
Clinical Surgery Pearls
Q 19. What are the manifestations of lymphangioma?
• Skin vesicles noticed by parent (may be clear
or brown or black) as a result of the contained
clotted blood—0.5 to 3 to 4 mm in diameter
• Some times the vesicles leak clear fluid
• May present with infected vesicles and pain
• The number and extent ofthe vesiclesincreases
with age
• Multiple small lesions may notfluctuate and feel
soft and spongy (one or two large cysts show
fluctuation, fluid thrill and translucency)
• It may or may not be compressible
• When there isinfection the regional nodes may
be enlarged.
Q 20. What are the classical sites of lymphangioma?
Classical sites of lymphangioma
• Junction of the limb and neck
• Junction of the limb and trunk
• Around the shoulder
• Axilla
• Buttock
• Groin.
Q 21. What is lymphangioma circumscriptum?
When the lesion islocalized to a region like buttocks
or side of the thigh it is called lymphangioma
circumscriptum.
Q 22. What is Vin rose patch?
It is a congenital intradermal vascular abnormality
in which mild dilatation of the vessels in the
subpapillary dermal plexus gives the skin a pale
pink color. It is associated with other vascular
abnormalities like extensive hemangioma,
arteriovenous fistulae and lymphedema. It can
occur anywhere and causes no symptoms.
Q 23. What is Campbell de Morgan spot?
It is a bright red well-defined spot caused by the
collection of dilated capillaries fed by a single or
cluster of arterioles. They are usually seen in older
age group above 45. One or more spots and some
times a cluster may be seen. The usual site is upper
half of the trunk and rarely in the limbs and face.
They look like drops of dark red paint.
Q 24. What is spider nevus?
It is a solitary dilated skin arteriole, with visible
radiating branches. They are usually associated
with chronic liver disease and tumors producing
estrogens. They appear on the upper half of the
trunk, the face and the arms. They fade completely
when compressed and refill as soon as the pressure
is released.
Q 25. What is the age group for venous malformation?
The venous malformation may present at birth,
childhood or adolescence.
Q 26. What is the clinical presentation of
arteriovenous fistula?
• In early stages a pink stain and increased local
temperatureofthe surroundingskin are the only
signs
• Gradually distended veins occur and a thrill
can be felt (followed by audible bruit). When
varicose vein and skin discoloration over the
lateral aspect of the thigh are present suspect
congenital AV fistula
• Extensive dilated tortuous veins which are
pulsatile (arterialized veins) are seen
• Limb hypertrophy and local gigantism will occur
• Blood is diverted from the arterial side to the
venous side resulting in distal hypoxia.
Congenital Arteriovenous Fistula/Hemangioma/Compressible Swelling
429
Q 27. What is the commonest site for congenital
AV fistula?
• The lower extremity is the most common site
• Upper extremity
• CongenitalAV fistula in relation to the superficial
temporal artery
• Congenital AV fistula in relation to the occipital
artery.
Q 28. What are the signs of congenital AV fistula?
The following are the signs of congenital AV fistula
• Port wine discoloration and varicose veins—
Suspect AV fistula.
• Local gigantism—Congenital arteriovenous
fistula in the young patient will produce increase
length and girth of the limb (the local gigantism
may be associated with scoliosis).
• The lower limb is apparently warmer and moist
than the unaffected side (in the case of head and
neck the affected side will be warmer).
• Palpable continuousthrill in the dilated vessels.
• Continuous machinery murmur with systolic
accentuation is obtained when the stethoscope
is applied to the arterialized veins.
• Collapsing arterial pulse.
• Bradycardiac signof Branham andNicolodona—
Digital occlusion of the main artery to the limb
is followed by bradycardia indicating that
considerable volume of blood is being short
circuited.
• Leg ulceration due to hypoxia—These ulcers are
known as hot ulcers (because the surrounding
skin feels warmerthan normal).These ulcers are
very painful.
Q 29. What are the causes for acquired AV fistula?
• Trauma to the vessels
• Iatrogenic AV fistula (for dialysis).
Q 30. What are the differences between congenital
and acquired AV fistula?
• The arteriovenous communications are
innumerable in congenital
• The communications are one ortwo in acquired
• Congenital is difficult to treat
• Acquired is easily treated.
Q 31. What is Cirsoid aneurysm?
They are nothing but congenital arteriovenous
malformations in relation to the superficial temporal
artery or occipital artery. They may have intracranial
extensions. It is very difficult to treat such lesions.
Q 32. What are the investigations in a case of
vascular malformation?
Color Doppler and MR angiography are the two
most useful investigations in the case of arteriovenous fistula.
• Laboratory investigations—No laboratory
investigation can differentiate hemangioma from
vascular malformation. However, estimation of
fibroblastic growth factor secreted in the urine
of patients with hemangioma may be useful.
• Duplex scanning—It is a useful noninvasive
investigation.
• Tc-99 labeled human albumin is injected intraarteriallyproximaltotheAVfistulaandmeasuring
the radioactivity in the lungs with a gamma
camera is a useful investigation for arteriovenous
fistula (< 3% passto the lungs normally).
• CT scanning with contrast enhancement
• MRI—Superior to CT (high and low flow lesions
can be identified).
• Magnetic resonance angiogram—This will
accurately distinguish hemangioma from
vascular malformations. This is the most useful
imaging study of choice.
430
Clinical Surgery Pearls
• Angiography—It is not routinely done unless
embolization is required prior to surgery.
Q 33. What is the treatment of hemangioma?
Treatment of hemangioma
• Most hemangiomas do not require any treatment
because they resolve spontaneously
• Avoid local trauma
• Parents are directto give local pressure in the event
of bleeding
• Corticosteroids (both systemic and intralesional)
• Interferon a2a - used for life-threatening complications (interferon is an inhibitor of angiogenesis)
• Lasers—Used to treat residual telangiectatic spots
in after involution
• Surgical excision—It is indicated when complications occur and also in the case of eyelids.
Q 34. What are the indications for corticosteroids?
• Life-threatening complications—Airway
obstruction, bleeding
• Large facial hemangiomas
• Platelet trapping syndrome.
Q 35. What is the dose and duration of therapy?
Prednisolone 2 mg/kg/day are used orally. The
treatment is continued for several months until the
tumor is in its involuting phase.
Q 36. What is intralesional steroid therapy?
• This is used for localized facial hemangiomas
• Triamcinolone—3 to 5 mg/kg/procedure is
injected directly into the lesion with a 26 gauge
needle.The volume should not exceed 1 mL per
injection.
Q 37. What are the management options in
venous malformation?
Management options in venous malformation
• Elastic support
• Lowdoseaspirinforpreventingepisodic thrombosis
• Sclerotherapy—sodium tetradecyl sulphate
injection locally followed by pressure
• Laser therapy
• Surgical treatment.
Q 38. What are the management options for
arteriovenous fistula?
Management options for arteriovenous fistula
Emergency management of bleeding
• Emergency ligation of feeding vessel or surgical
packing—this will give temporary control (always
recurrence should be anticipated and the recurrence
is too difficult to treat owing to the recruitment of
multiple new channels)
• Transcatheter embolisation
• Management of cardiac failure
Elective treatment
• Preoperative embolization followed by surgical
excision (The resectability rate is < 20%)
• The embolization should be done as near to the
time of surgery as is feasible.
Q 39. What are the materials used for embolization?
Materials used for embolization
• Plastic particles
• Foam pledgets
• Stainless steel coils
• Polymerizing adhesives.
Contd...
Congenital Arteriovenous Fistula/Hemangioma/Compressible Swelling
431
• Ethanol - polyvinylalcoholfoamparticlesareavailable
in graded sizesfrom 50–1000 micrometers diameter
• Sclerosing agents
• Acrylic adhesives.
Q 40. What is Klippel-Trenaunay syndrome?
They are combined vascular malformations
(combined in the sense that they involve more than
one type of channel). In this case, it is a combined
capillary lymphovenous malformation. It is a slow
flow lesion characterized by the following lesions:
Klippel-Trenaunay syndrome
• Geographic capillary stain
• Venous anomaly ofthe superficial and deep system
• Lymphatic abnormality
• Over growth of soft tissues and bones.
Contd... Q 41. What is Parkes-Weber syndrome?
This is a combined capillary and arteriovenous
malformation. It is a flat blue lesion characterized
by:
• Flat pink, (warm) stain
• Underlying multiple AV connections
• Venous malformation
• Tissue overgrowth.
Q 42. What is Maffucci syndrome?
The association of following constitutes Maffucci
syndrome:
Maffucci syndrome
• Multiple enchondromas
• Venous malformations
• Bony deformities.
42 Unilateral Lower Limb Edema
Case
Case Capsule
A 45-year-old male patient presents with swelling
of the left lower limb of 3 years duration. The
inguinal lymph nodes are enlarged, firm, discrete
and mobile. There is history of recurrent attacks
of fever, chills and pain in the affected limb.
The first episode of such attack started in early
adult life. In the initial stages the swelling used
to disappear in between the attacks of fever and
chills. The frequency of such attack is increasing
and the edema is persistent nowadays. The
swelling does not reverse on elevation of the limb.
The skin is thickened and some warty nodules
are seen in the lower part of the leg anteriorly.
The limb has attained enormous size in the last
few months and it is now interfering with mobility
of the limb and routine activities of the patient.
The edema is seen involving the dorsum of foot
and toes. On interrogation, it was found that he
is coming from a filarial belt. There is no history
of surgery and irradiation. Examination of the
genitalia revealed hydrocele of the TV sac on
left side. Abdominal examination is normal so
also the digital rectal examination. There is no
evidence of edema of the contralateral lower limb
and upper limbs.
Checklist for history
• History of geographical location from where the
patient is coming (endemic place for filariasis)
• Onset and duration of edema (congenital,
adolescence, or adulthood)
• History of injuries, wounds, abrasions, fissuring
of the skin, eczema and paronychia
• History of recurrent attacks of fever, chills and
painful swellings
• Whether the swelling is initially reversible or not
• History of tumors of the pelvic floor, prostate
cancer, etc.
• History of surgical dissection of lymph nodes
• History of radiation therapy for malignant tumors
• History of podoconiosis (cutaneous absorption of
mineral particles)
• History of venous diseases, venous surgery and
venous thrombosis
• History of Hansen’s disease
• History of Leishmaniasis
• History of cardiac diseases
• History of hepatic and nutritional disorders
• History of amyloidosis.
Checklist for examination
• Decide whether the edema is unilateral or bilateral
• Look carefully for thin red and tender streaks on
the skin suggestive of lymphangitis
Contd...
Unilateral Lower Limb Edema
433
• Check whether the edema is pitting or nonpitting
• Decide whether it is affecting the entire limb
or localized to the ankle region or affecting the
toes—ankle region is affected in DVT, entire
limb is affected in iliac vein occlusion
• Decide whether the skin is normal or
hyperkeratotic with nodules (hyperkeratotic
skin is seen in lymphedema)
• Examine for draining lymph nodes (lymph
nodes are enlarged in secondary lymph edema,
but not in DVT)
• Examine the genitalia to rule out hydrocele
and edema affecting the genitalia (both are
seen in lymphatic filariasis)
• Examine the breasts in females to rule out
edema of the breast (seen in filariasis)
Contd...
• Whenthere is evidenceof superficial thrombophlebitis—If the episodes are transient,
migrate and affect the arms in age group above
45 years suspect occult visceral carcinoma and
examine the abdomen to rule out Ca
• Examine the venous system to rule out chronic
venous insufficiency
• Examine the whole patient to rule out cardiac
causes and renal causes for edema.
Contd...
Contd...
434
Clinical Surgery Pearls
• Secondary lymphedema—Lymphatic filariasis
– Other infections: Tuberculosis, lymphogranuloma inguinale
– Tumors of the pelvic floor (prostate cancer)
– Surgical dissection of lymph nodes (block
dissection)
– Radiation therapy for malignant tumors
– Podoconiosis (cutaneous absorption of
mineral particles).
• Lymphedemasecondarytocongenitalvascular
anomalies
– Lymphatic angiodysplasia syndrome
– Klippel-Trenaunay syndrome
– Hyperstomy syndrome.
2. Lower limb edema due to venous causes:
• Chronic venous insufficiency
• Deep vein thrombosis
• Post-thrombotic syndrome
• Intravenous drug use
• Phlegmasia alba dolens (white leg or milk
leg)
• Phlegmasia cerulea dolens.
3. Cellulitis
4. Endocrine disease—pretibial myxoedema
5. Immobility and dependency—Dependency
edema (seen in cases of paralysis)
6. Hansen’s disease
7. Dermal leishmaniasis
8. Mycetoma
9. Systemic causes
10. Factitious—Self harm.
Q 4. What is edema?
Edema represents an imbalance between capillary
filtration and lymphatic drainage (the role of
lymphatics in the development of edema is
substantial. This does not mean that all edemas are
lymphedemas).
Q 1. What is your diagnosis?
Lymphatic filariasis with lymphedema.
Q 2. What are your points in favor of your
diagnosis?
• Patient gives history of recurrent fever, chills and
pain in the affected limb
• The edema is affecting the toes (a point in favor
of lymphedema)
• The edema is nonpitting
• The swelling does not reverse on elevation of
the limb
• The skin is thickened and warty nodules are seen
• The regional nodes are enlarged
• Patient is coming from an endemic area for
filariasis.
Q 3. What are the differential diagnoses?
1. Lower limb edema from lymphatic causes:
• Primary lymphedema
– lymphedema congenita
– lymphedema praecox: 2 to 35 years
(sporadic or familial). The familial is
called Meige’s disease.
– lymphedema tarda: After 35 years
(associated with obesity—the nodes
replaced with fibrofatty tissue).
• Familial lymphoedema (Milroy’s disease) —
familial form of congenital lymphedema
Two main types of familial (hereditary)
lymphedema are recognized—Nonne-milroy
(type I) and Letessier-meige (type II). The
incidence is 1 in 6000 live births and probably
inherited as autosomal dominant with
incomplete penetrance seen in chromosome
IV. In Meige’s disease lymphedema develops
between puberty and middle age.
Unilateral Lower Limb Edema
435
• Subfascialplexusin the muscular compartments
• The lymphaticsfinally drain to the lymph nodes.
Q 9. What is the WHO grading of lymphedema?
WHO grading of lymphedema of the limbs (1992)
Grade I – Pitting edema reversible on elevation
of the affected limb
Grade II – Pitting or nonpitting edema which does
not reverse on elevation of the affected
limb, and there are no skin changes
Grade III – Nonpitting edema that is not reversible,
with thickening of skin
Grade IV – Nonpitting edema that is not reversible,
with thickening of skin along with
nodular or warty excrescences—the
stage of elephantiasis.
Q 10. What are the disadvantages of this grading?
• Itdoesnotdifferentiatetheseverityofskinchanges
• Does not denote the magnitude of disability.
Q 11. What is the recent modification by the WHO?
Recent modification of WHO staging of lymphedema
Stage 1 – Same features as in grade I above
Stage 2 – Same features as in grade II above
Stage 3 – Presence of shallow skin folds
Stage 4 – Presence of knobs or nodules
Stage 5 – Presence of deep skin folds
Stage 6 – Warty changes in the skin
Stage 7 – Patient unable to move around due to
enormous size of the swelling.
Q 12. What is Brunner’s grading?
Grade
(Brunner)
Clinical features
Subclinical
(latent)
There is excess interstitial fluid and
histological abnormalities in lymphatics
and lymph nodes, but no clinically
apparent lymphedema
Q 5. How the lymphatic system is formed and what
is the function of lymphatic system?
It forms part of the microcirculation that helps to
return macromolecules like proteins, cell debris
and other particulate matter and excess fluid from
the interstitial spaces to the venous system via large
lymph vessels. The initial lymphatic capillaries in
the skin originate as blind ended tubes formed
by a single layer of endothelial cells with spaces in
between for the entry of fluid and large molecules.
They join to form larger vessel which have smooth
muscles in their walls. The flow of lymph is
unidirectional because of the presence of valves
situated every 2 to 12 mm distance. The larger
vessels are having smooth muscle in their walls.
Gentle massage of the skin stimulates contractions.
Lymphatics are responsible for resorption of 10 to
20% of the tissue fluid. Two to four liters of lymph
with 70 to 200 gm of protein pass daily into the
systemic circulation.
Q 6. What are the factors responsible for lymph flow?
• Contraction of muscles
• Pressure exerted by arterial pulsations
• Increase in fluid volume
• Elevation of the limb.
Q 7. What are the possible mechanisms for
lymphedema?
1. When there is increased load of interstitial
fluid—high output failure.
2. When the lymph vessels are absent, or abnormal
or damaged due to acquired causes—low
output failure.
Q 8. In which plane you get the lymphatics?
In the limbs they form:
• Superficial and deep plexus in the dermis—
they drain to the subcutaneous lymph vessels
following the course of superficial veins Contd...
436
Clinical Surgery Pearls
I Edema pits on pressure and swelling
largely or completely disappears on
elevation and bed rest
II Edema does not pit and does not
significantly reduce upon elevation
III Edema is associated with irreversible skin
changes, i.e. fibrosis, papillae
Q 13. What is the cause for primary lymphedema?
It is a congenital pathology affecting the lymphatic
channels in the form of:
• Agenesis of lymphatics(Aplasia) – lymphedema
congenita
• Hypoplasia— the commonest variety—
lymphedema praecox and tarda
• Hyperplasia—lymphaticsareenlarged,increased
in number and tortuous
• Lymphangiectasia.
Q 14. What are the clinical subtypes of primary
lymphedema?
The clinical subtypes are:
1. Congenital lymphedema
– which appears shortly after birth
2. Lymphedema praecox
– which starts during puberty
3. Lymphedema tarda
– which usually starts in the 3rd decade.
Q 15. What are the clinical syndromes associated
with primary lymphedema? (PG)
Syndromes associated with primary lymphedema
• Turner syndrome – XO karyotype
• Klinefelter syndrome – XXY
• Down’s – Trisomy 21
• Noonan syndrome
• Yellow nail syndrome
• Intestinal lymphangiectasia.
Q 16. What is the commonest cause for lymphedema?
The commonest cause is always secondary. The
commonest cause for secondary lymphedema
worldwide is lymphatic filariasis. For the other causes
for secondary lymphedema see answer of Q 3.
Q 17. What are the other cause for secondary
lymphoedema?
1. Malignant diseases – Lymph node metastasis
– Lymphoma
– Pressure from big tumors
– Infiltrating tumors
2. Traumatic damage – Secondary to
lymphadenectomy
(block dissection)
– Radiation to the lymph
node area
– Burns
– Large circumferential
wounds
– Scarring
– Varicose vein surgery:
Vein harvesting
3. Infections – Lymphadenitis
– Tuberculosis
– Cellulitis
– Other inflammatory
conditions like
rheumatoid arthritis and
sarcoidosis.
Q 18. What is acute dermatolymphangioadenitis
(ADLA)? (PG)
All cases of lymphedema whether primary
or secondary are prone for acute dermatolymphangioadenitis and the manifestations are:
clinical surgery pearls
clinical surgery pearls 3rd edition pdf
clinical surgery pearls 3rd edition pdf free download
clinical surgery pearls dayananda babu pdf
clinical surgery pearls pdf
clinical surgery pearls pdf google drive
clinical surgery pearls latest edition
clinical surgery pearls 2nd edition pdf free download
clinical surgery pearls pdf free download
clinical surgery pearls 3rd edition free pdf
clinical surgery pearls and co
clinical surgery pearls and beauty
clinical surgery pearls and skin
clinical surgery pearls clinic
clinical surgery pearls canada
clinical surgery pearls course
clinical surgery pearls clinical
clinical surgery pearls clinical trials
clinical surgery pearls dayananda babu pdf free download
clinical surgery pearls pdf download
clinical surgery pearls hotel
clinical surgery pearls hospital
clinical surgery pearls harvard
clinical surgery pearls harris
clinical pearls surgery
clinical surgery pearls journal
clinical surgery pearls japan
clinical surgery pearls jewellery
clinical surgery pearls jewelry
clinical surgery pearls kit
clinical surgery pearls ksa
clinical surgery pearls ks
clinical surgery pearls meaning
clinical surgery pearls mdpi
clinical surgery pearls medical
clinical surgery pearls md
clinical surgery pearls mcgill
clinical surgery pearls near me
clinical surgery pearls ncbi
clinical surgery pearls nursery
general surgery pearls of wisdom pdf
clinical surgery pearls qatar
clinical surgery pearls questions
clinical surgery pearls quiz
clinical surgery pearls review
clinical surgery pearls reviews
clinical surgery pearls spain
clinical surgery pearls spa
clinical surgery pearls sc
clinical surgery pearls st
clinical surgery pearls test
clinical surgery pearls therapy
clinical surgery pearls thesis
clinical surgery pearls video
clinical surgery pearls vessel
clinical surgery pearls valley
clinical surgery pearls xl
clinical surgery pearls youtube
clinical surgery pearls yellow
clinical surgery pearls yahoo finance
clinical surgery pearls yahoo
clinical surgery pearls yugioh
clinical surgery pearls zambia
clinical surgery pearls zurich
clinical surgery pearls zone
clinical surgery pearls zanzibar
can clinical surgery pearls and co
can clinical surgery pearls and beauty
can clinical surgery pearls and skin
can clinical surgery pearls benefits
can clinical surgery pearls before and after
can clinical surgery pearls before swine
can clinical surgery pearls black
can clinical surgery pearls canada
can clinical surgery pearls clinic
can clinical surgery pearls cost
can clinical surgery pearls costco
can clinical surgery pearls co
can clinical surgery pearls experience
can clinical surgery pearls free
can clinical surgery pearls for sale
can clinical surgery pearls for dogs
can clinical surgery pearls guide
can clinical surgery pearls good
can clinical surgery pearls growth
can clinical surgery pearls good or bad
can clinical surgery pearls guidelines
can clinical surgery pearls hospital
can clinical surgery pearls harvard
can clinical surgery pearls how to use
can clinical surgery pearls inc
can clinical surgery pearls in india
can clinical surgery pearls in china
can clinical surgery pearls in canada
can clinical surgery pearls journal
can clinical surgery pearls jobs
can clinical surgery pearls job description
can clinical surgery pearls job
can clinical surgery pearls journals
can clinical surgery pearls kit
can clinical surgery pearls kopen
can clinical surgery pearls ksa
can clinical surgery pearls meaning
can clinical surgery pearls mdpi
can clinical surgery pearls medical
can clinical surgery pearls md
can clinical surgery pearls mcgill
can clinical surgery pearls near me
can clinical surgery pearls ncbi
can clinical surgery pearls nursery
can clinical surgery pearls online
can clinical surgery pearls of the world
can clinical surgery pearls quotes
can clinical surgery pearls questions
can clinical surgery pearls quiz
can clinical surgery pearls quora
can clinical surgery pearls review
can clinical surgery pearls reviews
can clinical surgery pearls reddit
can clinical surgery pearls test
can clinical surgery pearls thesis
can clinical surgery pearls uk
can clinical surgery pearls uses
can clinical surgery pearls us
can clinical surgery pearls use
can clinical surgery pearls up
can clinical surgery pearls video
can clinical surgery pearls valley
can clinical surgery pearls work
can clinical surgery pearls worth
can clinical surgery pearls worth it
can clinical surgery pearls with
can clinical surgery pearls works
can clinical surgery pearls xl
can clinical surgery pearls xray
can clinical surgery pearls youtube
can clinical surgery pearls yellow
can clinical surgery pearls you
can clinical surgery pearls zurich
can clinical surgery pearls zambia
can clinical surgery pearls zanzibar
how clinical surgery pearls are
how clinical surgery pearls are made
how clinical surgery pearls benefits
how clinical surgery pearls before and after
how clinical surgery pearls canada
how clinical surgery pearls cost
how clinical surgery pearls cancer
how clinical surgery pearls come from
how clinical surgery pearls experience
how clinical surgery pearls experiment
how clinical surgery pearls free
how clinical surgery pearls foundation
how clinical surgery pearls good
how clinical surgery pearls grow
how clinical surgery pearls goodreads
how clinical surgery pearls good or bad
how clinical surgery pearls goods
how clinical surgery pearls have
how clinical surgery pearls harvard
how clinical surgery pearls inc
how clinical surgery pearls is
how clinical surgery pearls journal
how clinical surgery pearls jewellery
how clinical surgery pearls job description
how clinical surgery pearls journals
how clinical surgery pearls jewelry
how clinical surgery pearls kit
how clinical surgery pearls ksa
how clinical surgery pearls ks
how clinical surgery pearls knowledge
how clinical surgery pearls meaning
how clinical surgery pearls mean
how clinical surgery pearls means
how clinical surgery pearls made
how clinical surgery pearls make
how clinical surgery pearls near me
how clinical surgery pearls needed
how clinical surgery pearls norway
how clinical surgery pearls open
how clinical surgery pearls on
how clinical surgery pearls out
how clinical surgery pearls opened
how clinical surgery pearls of the world
how clinical surgery pearls questions
how clinical surgery pearls quiz
how clinical surgery pearls quotes
how clinical surgery pearls quora
how clinical surgery pearls quizlet
how clinical surgery pearls review
how clinical surgery pearls start
how clinical surgery pearls soap
how clinical surgery pearls started
how clinical surgery pearls test
how clinical surgery pearls thesis
how clinical surgery pearls use
how clinical surgery pearls uses
how clinical surgery pearls up
how clinical surgery pearls used
how clinical surgery pearls video
how clinical surgery pearls valley
how clinical surgery pearls work
how clinical surgery pearls works
how clinical surgery pearls worth
how clinical surgery pearls worked
how clinical surgery pearls worth it
how clinical surgery pearls xl
how clinical surgery pearls xray
how clinical surgery pearls youtube
how clinical surgery pearls you
how clinical surgery pearls yellow
how clinical surgery pearls yeast
how clinical surgery pearls zurich
how clinical surgery pearls zap
how clinical surgery pearls zundert
how to do clinical surgery pearls
which clinical surgery pearls are best
which clinical surgery pearls are made
which clinical surgery pearls are the same
which clinical surgery pearls benefits
which clinical surgery pearls before and after
which clinical surgery pearls better
which clinical surgery pearls canada
which clinical surgery pearls cost
which clinical surgery pearls come from
which clinical surgery pearls everyday
which clinical surgery pearls end
which clinical surgery pearls fit
which clinical surgery pearls foundation
which clinical surgery pearls found
which clinical surgery pearls fellow
which clinical surgery pearls fellows
which clinical surgery pearls good
which clinical surgery pearls grow
which clinical surgery pearls have
which clinical surgery pearls is best
which clinical surgery pearls is good
which clinical surgery pearls journal
which clinical surgery pearls jewellery
which clinical surgery pearls joint
which clinical surgery pearls killed
which clinical surgery pearls mean
which clinical surgery pearls made
which clinical surgery pearls means
which clinical surgery pearls make
which clinical surgery pearls needed
which clinical surgery pearls near me
which clinical surgery pearls need
which clinical surgery pearls open
which clinical surgery pearls on
which clinical surgery pearls offer
which clinical surgery pearls of the world
which clinical surgery pearls on a plane
which clinical surgery pearls quora
which clinical surgery pearls quiz
which clinical surgery pearls quality
which clinical surgery pearls questions
which clinical surgery pearls quote
which clinical surgery pearls review
which clinical surgery pearls real
which clinical surgery pearls replacement
which clinical surgery pearls reviews
which clinical surgery pearls support
which clinical surgery pearls start
which clinical surgery pearls started
which clinical surgery pearls test
which clinical surgery pearls the same
which clinical surgery pearls use
which clinical surgery pearls uses
which clinical surgery pearls valley
which clinical surgery pearls work
which clinical surgery pearls works
which clinical surgery pearls worth it
which clinical surgery pearls worth
which clinical surgery pearls xl
which clinical surgery pearls x ray
which clinical surgery pearls you
which clinical surgery pearls youtube
which clinical surgery pearls zurich
which clinical surgery pearls zap
which clinical surgery pearls zen
surgical examination
asmbs clinical pearls for emergency care of the bariatric surgery patient
clinical pearls for emergency care of the bariatric surgery patient
surgery examination
kaplan obstetrics and gynecology videos
طبيب سوداني باثولوجي
best of clinical surgery pearls
best and clinical surgery pearls
clinical surgery pearls شرح
clinical surgery pearls مترجم
clinical surgery pearls arabic
clinical surgery pearls download
clinical surgery pearls en arabe
clinical surgery pearls egybest
clinical surgery pearls greek
clinical surgery pearls gel شرح
clinical surgery pearls how to use in hindi
clinical surgery pearls hindi
clinical surgery pearls in hindi
clinical surgery pearls in شرح
clinical surgery pearls in arabic
clinical surgery pearls kurdish
clinical surgery pearls plus شرح
clinical surgery pearls review شرح
clinical surgery pearls uses in hindi
clinical surgery pearls uses in urdu
clinical surgery pearls us شرح
clinical surgery pearls white شرح
clinical surgery pearls x شرح
clinical surgery pearls xl شرح
can clinical surgery pearls arabic
can clinical surgery pearls download
can clinical surgery pearls egybest
can clinical surgery pearls greek
can clinical surgery pearls gel شرح
can clinical surgery pearls how to use in hindi
can clinical surgery pearls in hindi
can clinical surgery pearls in شرح
can clinical surgery pearls in arabic
can clinical surgery pearls like شرح
can clinical surgery pearls pdf
can clinical surgery pearls para que sirve
can clinical surgery pearls turkish
can clinical surgery pearls uses in hindi
can clinical surgery pearls uses in urdu
can clinical surgery pearls white شرح
can clinical surgery pearls x شرح
can clinical surgery pearls yapımı
how clinical surgery pearls arabic
how clinical surgery pearls download
how clinical surgery pearls does شرح
how clinical surgery pearls egybest
how clinical surgery pearls greek
how clinical surgery pearls gel شرح
how clinical surgery pearls hindi
how clinical surgery pearls how to use in hindi
how clinical surgery pearls in hindi
how clinical surgery pearls in hindi شرح
how clinical surgery pearls in شرح
how clinical surgery pearls in arabic
how clinical surgery pearls kurdish
how clinical surgery pearls like شرح
how clinical surgery pearls pdf
how clinical surgery pearls uses in hindi
how clinical surgery pearls uses in urdu
how clinical surgery pearls use in hindi
how clinical surgery pearls works شرح
how clinical surgery pearls work شرح
how clinical surgery pearls x شرح
how clinical surgery pearls zinc شرح
which clinical surgery pearls arabic
which clinical surgery pearls download
which clinical surgery pearls egybest
which clinical surgery pearls in hindi
which clinical surgery pearls pdf
which clinical surgery pearls quail
which clinical surgery pearls uses in hindi
which clinical surgery pearls use in hindi
which clinical surgery pearls uses in urdu
No comments:
Post a Comment
اكتب تعليق حول الموضوع