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Primary Nonneoplastic Cysts Overview

(Left) Gross pathology shows

an autopsied case of a colloid

cyst, sectioned in the coronal

plane through the foramen of

Monro. Note the gelatinousappearing lobulated cyst ﬈

with the fornices st straddling

the cyst. This patient died of

sudden obstructive

hydrocephalus. (Courtesy J.

Townsend, MD.) (Right) Gross

pathology of an autopsied

brain, seen from below, shows

a large arachnoid cyst of the

middle cranial fossa. The cyst

contained cerebrospinal fluid

within split layers of arachnoid

﬈. (Courtesy J. Townsend,

MD.)

(Left) Gross surgical specimen

of a sectioned dermoid cyst

shows the characteristic lining

of stratified squamous

epithelium plus intracystic

keratin debris ſt. Matted,

tangled hairs ﬊ are present

within the cyst, which

contained thick, greasy

sebaceous material when

sectioned. (Courtesy R.

Hewlett, MD.) (Right)

Microscopy of a typical

dermoid cyst shows squamous

epithelium ﬈ and sebaceous

glands ﬊ lining a cavity that

contains desquamated

keratinaceous debris.

(Left) Close-up view of an

epidermoid cyst shows the

cauliflower-like cyst surface

that is composed of nodular

masses of squamous

epithelium and pearly white

keratin. (Right) Autopsy

specimen shows a small,

gelatinous-appearing nodule

﬈ just anterior to the pons.

Ecchordosis physaliphora, a

notochordal remnant, grossly

appears similar to neurenteric

cyst. (Courtesy R. Hewlett,

MD.)

Brain: Pathology-Based Diagnoses: Neoplasms,

Cysts, and Disorders

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Primary Nonneoplastic Cysts Overview

Intracranial Cystic-Appearing Lesions

Extraaxial Intraaxial

Supratentorial Supratentorial

Midline Parenchymal

Pineal cyst Enlarged PVSs

Dermoid cyst Neuroglial cyst

Rathke cleft cyst Porencephalic cyst

Arachnoid cyst (suprasellar) Connatal, germinolytic cysts

Hippocampal sulcus remnants

Off midline Intraventricular

Arachnoid cyst (middle cranial fossa, convexity) Choroid plexus cysts

Epidermoid cyst Ependymal cyst

TACs (macroadenoma, meningioma) Colloid cyst

Sebaceous cyst (scalp)

Leptomeningeal cyst ("growing fracture")

Infratentorial Infratentorial

Midline Parenchymal

Neurenteric cyst Enlarged PVSs (dentate nuclei)

Arachnoid cyst

Off midline Intraventricular

Epidermoid (CPA) Epidermoid (4th ventricle, cisterna magna)

Arachnoid cyst (CPA) Cystic ("trapped") 4th ventricle

TACs (schwannoma, meningioma)

Nonneoplastic, noninfectious cystic brain lesions are classified by common anatomic locations. The 1st division is extra- vs. intraaxial, then supra- vs.

infratentorial. Extraaxial cysts are further subdivided into midline and off midline lesions. Intraaxial cysts are subdivided into parenchymal and

intraventricular. CPA = cerebellopontine angle; PVSs = perivascular (Virchow-Robin) spaces; TACs = tumor-associated cysts.

Intracranial Cysts by Type, Most Common Location(s)

Cyst Type Common Location(s)

Arachnoid cyst Middle cranial fossa, CPA, suprasellar cistern

Choroid fissure cyst Choroid fissure, between temporal horn and suprasellar cistern

Choroid plexus cyst Choroid plexus glomus

Colloid cyst Interventricular foramen/anterosuperior 3rd ventricle

Connatal cyst(s) Peri- or intraventricular, adjacent to frontal horn, body of lateral ventricle

Dermoid cyst Suprasellar, frontonasal (anteroinferior interhemispheric fissure)

Enlarged PVSs Basal ganglia, midbrain, cerebral white matter, dentate nuclei

Epidermoid cyst CPA

Ependymal cyst Lateral ventricle (atrium most common)

Germinolytic pseudocyst(s) Periventricular, subependymal along caudothalamic groove

Hippocampal sulcus remnants Hippocampus, just medial to lateral ventricle

Leptomeningeal cyst ("growing fracture") Parietal bone

Neurenteric cyst Prepontine at pontomedullary junction

Neuroglial cyst Frontal/temporal subcortical white matter, choroid fissure

Pineal cyst Pineal gland

Porencephalic cyst Cerebral hemispheres, adjacent to lateral ventricles

Rathke cleft cyst Suprasellar, intrasellar

Sebaceous (trichilemmal) cyst Scalp (dermis or subcutaneous tissues)

Tumor-associated cyst Between schwannoma, meningioma, macroadenoma, and brain

Cysts, and Disorders

Brain: Pathology-Based Diagnoses: Neoplasms,

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ular cysts are most often found in

the atria of the lateral ventricles and foramen of Monro. CPCs

are the most common of all intracranial neuroepithelial cysts,

occurring in up to 50% of autopsies. Most CPCs are actually

xanthogranulomas. Lipid accumulates in the choroid plexus

from degenerating &/or desquamating choroid epithelium.

CPCs are common incidental imaging findings in middle-aged

and older adults. They are usually bilateral and are often

multicystic. Most CPCs are small, measuring 2-8 mm in

diameter. They typically do not suppress completely on FLAIR

and may show moderately high signal intensity on DWI.

Ependymal cysts (ECs) are rare, benign, ependymal-lined cysts

of the lateral ventricles. Most ECs, even large ones, are

asymptomatic and incidental. EC patients presenting with

headache, seizure, &/or obstructive hydrocephalus have been

reported in the literature. They contain clear serous CSF-like

fluid secreted from ependymal cells. ECs typically follow CSF

on all sequences and suppress completely on FLAIR.

CCs occur almost exclusively in the foramen of Monro,

attached to the anterosuperior portion of the 3rd ventricular

roof. They are wedged into the foramen and are typically

straddled by the fornices. CCs are endodermal in origin and

contain viscous gelatinous material consisting of mostly

mucin. CCs may also contain blood degradation products,

foamy cells, and cholesterol crystals. Even relatively small CCs

may suddenly obstruct the foramen of Monro, causing acute

hydrocephalus. Occasionally brain herniation with rapid clinical

deterioration ensues. The imaging appearance of a welldelineated, hyperdense mass at the foramen of Monro on

NECT is virtually pathognomonic of a CC.

Infratentorial intraaxial cysts: Parenchymal infratentorial

cysts are rare; most are PVSs. The only common site is in and

around the dentate nuclei. Most are asymptomatic.

Occasionally, large PVSs occur in the pons and can be a rare

cause of cranial neuropathy.

Nonneoplastic, nonparasitic cysts in the 4th ventricle are

uncommon. The most common cause is not a true cyst but an

enlarged, "encysted" 4th ventricle. Infection or aneurysmal

subarachnoid hemorrhage may cause outlet foraminal

obstruction. When combined with superior obstruction near

the aqueduct, the 4th ventricle can become completely

encysted. Choroid plexus continues to produce CSF. With

egress blocked, the 4th ventricle enlarges. ECs can arise in the

4th ventricle, a much less common posterior fossa location

than the CPA. Some ECs are so similar to CSF that only FLAIR

and DWI permit distinction of an EC from CSF in an enlarged

but otherwise normal-appearing 4th ventricle.

Selected References

1. Aboud E et al: Giant intracranial epidermoids: is total removal feasible? J

Neurosurg. 1-14, 2015

2. Ali M et al: Exploring predictors of surgery and comparing operative

treatment approaches for pediatric intracranial arachnoid cysts: a case series

of 83 patients. J Neurosurg Pediatr. 1-8, 2015

3. Culver SA et al: A Case for conservative management: characterizing the

natural history of radiographically diagnosed Rathke cleft cysts. J Clin

Endocrinol Metab. 100(10):3943-8, 2015

4. Din NU et al: Symptomatic surgically treated non-neoplastic cysts of the

central nervous system: a clinicopathological study from Pakistan. J Coll

Physicians Surg Pak. 25(8):588-91, 2015

5. Kalani MY et al: Pineal cyst resection in the absence of ventriculomegaly or

Parinaud's syndrome: clinical outcomes and implications for patient

selection. J Neurosurg. 1-5, 2015

6. Lauretti L et al: Treatment of giant congenital cysts of the midline in adults:

report of two cases and review of the literature. Surg Neurol Int. 6(Suppl

13):S371-4, 2015

7. Bender B et al: MR imaging findings in colloid cysts of the sellar region:

comparison with colloid cysts of the third ventricle and Rathke's cleft cysts.

Acad Radiol. 20(11):1457-65, 2013

8. Osborn AG et al: Intracranial cysts: radiologic-pathologic correlation and

imaging approach. Radiology. 239(3):650-64, 2006

Brain: Pathology-Based Diagnoses: Neoplasms,

Cysts, and Disorders

158

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