Neurofibroma (NF): NFs are diffusely infiltrating, extraneural
tumors that consist of Schwann cells and fibroblasts. Solitary
scalp NFs occur. Multiple NFs or plexiform NFs occur as part of
NF1. NFs correspond histologically to WHO grade I. Plexiform
NFs may degenerate into malignant peripheral nerve sheath
tumors (MPNSTs). MPNSTs are graded from WHO II to IV, an
approach similar to sarcoma grading.
Lymphomas and Hematopoietic Tumors
Primary CNS Lymphoma (PCNSL): As a result of HAART
therapy in HIV/AIDS patients, as well as in other
immunocompromised individuals, PCNSLs are dramatically
increasing in prevalence. PCNSL can occur as both focal
parenchymal and intravascular tumor. PCNSL can be single or
multiple and is most commonly seen in the cerebral
hemispheres. More than 95% of PCNSLs are diffuse large Bcell lymphomas.
Intracranial germ cell tumors are morphologic homologues of
germinal neoplasms that arise in the gonads and extragonadal
sites. 80-90% occur in adolescents. Most occur in the midline
(pineal region, around the 3rd ventricle).
Craniopharyngioma: Craniopharyngioma is a benign (WHO
grade I), often partially cystic neoplasm that is the most
common nonneuroepithelial intracranial neoplasm in children.
It shows a distinct bimodal age distribution, with the cystic
adamantinomatous type seen mostly in children and a 2nd,
smaller peak in middle-aged adults. The less common papillary
type is usually solid and found almost exclusively in adults.
Miscellaneous sellar region tumors: Granular cell tumor of
the neurohypophysis is a rare tumor of adults that usually
arises from the infundibulum. Pituicytomas are glial
neoplasms of adults that also usually arise within the
infundibulum. Spindle cell oncocytoma of the
adenohypophysis is a nonendocrine neoplasm of the
adenohypophysis. These rare tumors are all WHO grade I.
Brain: Pathology-Based Diagnoses: Neoplasms,
Neoplasm Grade Neoplasm Grade Neoplasm Grade
Astrocytic Choroid plexus Neuronal, mixed neuronal-glial
Pilocytic astrocytoma I Choroid plexus papilloma I Gangliocytoma I
Pilomyxoid astrocytoma II Atypical choroid plexus papilloma II Ganglioglioma I
Subependymal giant cell astrocytoma I Choroid plexus carcinoma III DIG/DIA I
Pleomorphic xanthoastrocytoma II DNET I
Anaplastic astrocytoma III Pineal region Central neurocytoma II
Glioblastoma IV Pineocytoma I Extraventricular neurocytoma II
Gliosarcoma IV PPTID II-III Cerebellar liponeurocytoma II
Gliomatosis cerebri III, II-IV Pineoblastoma IV Paraganglioma (spinal cord) I
Papillary tumor of pineal region II-III Papillary glioneuronal tumor I
Oligodendroglial Rosette-forming glioneuronal
Oligodendroglioma II Ependymal
Anaplastic oligodendroglioma III Subependymoma I Other neuroepithelial
Oligoastrocytoma Variable Myxopapillary ependymoma I Astroblastoma
Ependymoma II Chordoid glioma of 3rd ventricle II
Embryonal Anaplastic ependymoma III Angiocentric glioma (angiocentric
Primitive neuroectodermal tumor IV
Atypical teratoid-rhabdoid tumor IV
Neoplasm Grade Neoplasm Grade Neoplasm Grade
Meningothelial Nonmeningothelial mesenchymal Primary melanocytic
Meningioma I Lipoma I Diffuse melanocytoma
Atypical meningioma II Liposarcoma Melanocytoma
Anaplastic/malignant meningioma III Chondroma I Malignant melanoma
Chondrosarcoma Meningeal melanomatosis
Hemangioblastoma I Osteosarcoma
Neoplasm Grade Neoplasm Grade Neoplasm Grade
Cranial and spinal nerve tumors Germ cell tumors Sellar region tumors
Schwannoma I Germinoma Craniopharyngioma I
Neurofibroma I Embryonal carcinoma Adamantinomatous
MPNST II-IV Yolk sac tumor Papillary
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