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CNS tumor

Authors: Eman Abdelzaher, M.D., Ph.D., Janice Seulgy Ahn, M.D., Jared T. Ahrendsen, M.D., Ph.D., Ethar Al-Husseinawi, M.B.Ch.B., Ph.D., Sanda Alexandrescu, M.D., Paul J. Biggs, M.D., Andrey Bychkov, M.D., Ph.D., Chunyu Cai, M.D., Ph.D., Momal Tara Chand, M.D., P.J. Cimino, M.D., Ph.D., Yanel De Los Santos, M.D., John DeWitt, M.D., Ph.D., Angela France, D.O., Jesse L. Kresak, M.D., William Kupsky, M.D., Nelli S. Lakis, M.D., M.Sc., Maria Martinez-Lage, M.D., Ashirwad Merve, M.B.B.S., Ph.D., Meaghan Morris, M.D., Ph.D., Nat Pernick, M.D., Michael Punsoni, M.D., Fausto J. Rodriguez, M.D., Maxwell Rollins, M.D., Federico Roncaroli, M.D., Katherine Schwetye, M.D., Ph.D., Nirupama Singh, M.D., Ph.D., Mariana Voudouri, M.D., Shih-Hsiu "Jerry" Wang, M.D., Ph.D., Anthony T. Yachnis, M.D., Irfan Yasin, M.B.B.S., Y. Albert Yeh, M.D., Ph.D., Rebecca Yoda, M.D., George Zanazzi, M.D., Ph.D.

Minor changes: 14 January 2022

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Editorial Board oversight: Chunyu Cai, M.D. (last reviewed December 2021), Debra Zynger, M.D. (last reviewed December 2020).

NOTE: Revisions are in progress based on the 2021 WHO classification.
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Table of contents
General:anatomy & histology-pineal gland books CNS-gross (pending) CNS-histology (pending) glioma overview grossing, features to report & staging larotrectinib (LOXO-101) WHO classification
Cysts:arachnoid cyst choroid plexus cyst colloid cyst dermoid cyst enterogenous cyst epidermoid cyst glioependymal / ependymal cyst meningeal cyst pineal cyst Rathke cleft cyst simple cyst synovial cyst syrinx
Gliomas, glioneuronal tumors, and neuronal tumors
Diffuse astrocytic and oligodendroglial tumors:diffuse astrocytic and oligodendroglial tumor-general WHO grading of gliomas diffuse astrocytic glioma, IDH wildtype, with molecular features of glioblastoma diffuse astrocytoma IDH mutant diffuse low grade glioma, MAPK pathway-altered (pending) gemistocytic astrocytoma IDH mutant diffuse astrocytoma IDH wildtype diffuse astrocytoma, NOS anaplastic astrocytoma, IDH mutant anaplastic astrocytoma, IDH wildtype anaplastic astrocytoma, NOS glioblastoma-giant cell glioblastoma, IDH mutant glioblastoma, IDH wildtype gliosarcoma oligodendroglioma, IDH mutant and 1p/19q codeleted oligodendroglioma, NOS anaplastic oligodendroglioma, IDH-mutant and 1p / 19q-codeleted anaplastic oligodendroglioma oligoastrocytoma, NOS and anaplastic oligoastrocytoma, NOS
Other astrocytic tumors:pilocytic astrocytoma pilomyxoid astrocytoma subependymal giant cell astrocytoma pleomorphic xanthoastrocytoma anaplastic pleomorphic xanthoastrocytoma (pending)
Pediatric-type diffuse high grade gliomas :diffuse midline glioma, H3 K27M mutant diffuse hemispheric glioma, H3 G34 mutant
Ependymal tumors:ependymoma clear cell ependymoma papillary ependymoma tanycytic ependymoma anaplastic ependymoma myxopapillary ependymoma subependymoma RELA fusion positive ependymoma
Other gliomas:angiocentric glioma astroblastoma chordoid glioma of the third ventricle
Neuronal and mixed neuronal-glial tumors:dysembryoplastic neuroepithelial tumor gangliocytoma & ganglioglioma anaplastic ganglioglioma (pending) dysplastic gangliocytoma of the cerebellum desmoplastic infantile astrocytoma / ganglioglioma papillary glioneuronal tumor rosette forming glioneuronal tumor diffuse leptomeningeal glioneuronal tumor central neurocytoma extraventricular neurocytoma cerebellar liponeurocytoma paraganglioma
Embryonal tumors
Medulloblastoma:WNT activated (pending) SHH activated medulloblastoma desmoplastic / nodular medulloblastoma
Embryonal tumor with multilayered rosettes:embryonal tumor with abundant neuropil and true rosettes, C19MC altered ependymoblastoma, C19MC altered medulloepithelioma, C19MC not altered
Other embryonal tumors:atypical teratoid / rhabdoid tumor neuroblastoma ganglioneuroblastoma CNS embryonal tumor, NOS (pending)
Choroid plexus tumors:choroid plexus tumors (papilloma, atypical papilloma, carcinoma)
Pineal tumors:papillary tumor of pineal region pineoblastoma pineocytoma pineal parenchymal tumor of intermediate differentiation
Meningeal tumors:meningioma WHO grading of meningiomas anaplastic meningioma atypical meningioma chordoid meningioma clear cell meningioma papillary meningioma rhabdoid meningioma secretory meningioma
Germ cell tumors:choriocarcinoma embryonal carcinoma germinoma teratoma yolk sac tumor
Sellar region tumors:craniopharyngioma-adamantinomatous papillary craniopharyngioma pituicytoma pituitary adenoma pituitary carcinoma pituitary hyperplasia spindle cell oncocytoma
Lymphoma & histiocytic tumors:histiocytic tumors immunodeficiency associated (pending)
Other tumors:hemangioblastoma melanocytic tumors / melanoma meningioangiomatosis metastases paraneoplastic syndromes schwannoma solitary fibrous tumor
CNS tumor stains: ATRX BRAF V600E GFAP IDH1 (R132H) NeuN NFP / neurofilament Olig2 SSTR2A synaptophysin

Disorders from other chapters: primary CNS lymphoma

Superpages: entire chapterimagesvirtual slides
Index (Alphabetical table of contents)
A-E:anaplastic astrocytoma, IDH mutant anaplastic astrocytoma, IDH wildtype anaplastic astrocytoma, NOS anaplastic ependymoma anaplastic ganglioglioma (pending) anaplastic meningioma anaplastic oligodendroglioma anaplastic oligodendroglioma, IDH-mutant and 1p / 19q-codeleted anaplastic pleomorphic xanthoastrocytoma (pending) anatomy & histology-pineal gland angiocentric glioma arachnoid cyst astroblastoma atypical meningioma atypical teratoid / rhabdoid tumor central neurocytoma cerebellar liponeurocytoma chordoid glioma of the third ventricle chordoid meningioma choriocarcinoma choroid plexus cyst choroid plexus tumors (papilloma, atypical papilloma, carcinoma) clear cell ependymoma clear cell meningioma CNS embryonal tumor, NOS (pending) CNS-gross (pending) CNS-histology (pending) colloid cyst craniopharyngioma-adamantinomatous dermoid cyst desmoplastic / nodular medulloblastoma desmoplastic infantile astrocytoma / ganglioglioma diffuse astrocytic and oligodendroglial tumor-general diffuse astrocytic glioma, IDH wildtype, with molecular features of glioblastoma diffuse astrocytoma IDH mutant diffuse astrocytoma IDH wildtype diffuse astrocytoma, NOS diffuse hemispheric glioma, H3 G34 mutant diffuse leptomeningeal glioneuronal tumor diffuse low grade glioma, MAPK pathway-altered (pending) diffuse midline glioma, H3 K27M mutant dysembryoplastic neuroepithelial tumor dysplastic gangliocytoma of the cerebellum embryonal carcinoma embryonal tumor with abundant neuropil and true rosettes, C19MC altered enterogenous cyst ependymoblastoma, C19MC altered ependymoma epidermoid cyst extraventricular neurocytoma
F-N:gangliocytoma & ganglioglioma ganglioneuroblastoma gemistocytic astrocytoma IDH mutant germinoma glioblastoma, IDH mutant glioblastoma, IDH wildtype glioblastoma-giant cell glioependymal / ependymal cyst glioma overview gliosarcoma grossing, features to report & staging hemangioblastoma histiocytic tumors immunodeficiency associated (pending) larotrectinib (LOXO-101) medulloblastoma medulloepithelioma, C19MC not altered melanocytic tumors / melanoma meningeal cyst meningioangiomatosis meningioma metastases myxopapillary ependymoma neuroblastoma
O-Z:oligoastrocytoma, NOS and anaplastic oligoastrocytoma, NOS oligodendroglioma, IDH mutant and 1p/19q codeleted oligodendroglioma, NOS papillary craniopharyngioma papillary ependymoma papillary glioneuronal tumor papillary meningioma papillary tumor of pineal region paraganglioma paraneoplastic syndromes pilocytic astrocytoma pilomyxoid astrocytoma pineal cyst pineal parenchymal tumor of intermediate differentiation pineoblastoma pineocytoma pituicytoma pituitary adenoma pituitary carcinoma pituitary hyperplasia pleomorphic xanthoastrocytoma Rathke cleft cyst RELA fusion positive ependymoma rhabdoid meningioma rosette forming glioneuronal tumor schwannoma secretory meningioma SHH activated simple cyst solitary fibrous tumor spindle cell oncocytoma subependymal giant cell astrocytoma subependymoma synovial cyst syrinx tanycytic ependymoma teratoma WHO classification WHO grading of gliomas WHO grading of meningiomas WNT activated (pending) yolk sac tumor
CNS tumor books

(IARC): 2016

Asa: 2011

Gupta: 2016

IARC: 2017

Kleinschmidt-DeMasters: 2016

Love: 2015

Perry: 2017

Rodriguez: 2016



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