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2000 WHO Classification
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World Health Organization Classification of Tumors of the Nervous System
From:
Kleihues P, Cavenee WK, eds. 
Pathology and genetics of tumours of the nervous system

World Health Organization classification of tumours
Lyon, France: IARC Press, 2000



Contents



Tumors of Neuroepithelial Tissue

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Astrocytic Tumors
1 9400/3(*) Diffuse Astrocytoma
(Variant) 9420/3 Fibrillary Astrocytoma
(Variant) 9410/3 Protoplasmatic Astrocytoma
(Variant) 9411/3 Gemistocytic Astrocytoma
2 9401/3 Anaplastic Astrocytoma
3 9440/3 Glioblastoma
(Variant) 9441/3 Giant Cell Glioblastoma
(Variant) 9442/3 Gliosarcoma
4 9421/1 Pilocytic Astrocytoma
5 9424/3 Pleomorphic Xanthoastrocytoma
6 9384/1 Subependymal Giant Cell Astrocytoma



Oligodendroglial Tumors
7 9450/3 Oligodendroglioma
8 9451/3 Anaplastic Oligodendroglioma



Mixed Gliomas
9 9382/3 Oligoastrocytoma
10 9382/3(**) Anaplastic Oligoastrocytoma



Ependymal Tumors
11 9391/3 Ependymoma
(Variant) 9391/3 Cellurar
(Variant) 9393/3 Papillary
(Variant) 9391/3 Clear Cell
(Variant) 9391/3 Tanycytic
12 9392/3 Anaplastic Ependymoma
13 9394/1 Myxopapillary Ependymoma
14 9383/1 Subependymoma



Choroid Plexus Tumors
15 9390/0 Choroid Plexus Papilloma
16 9390/3 Choroid Plexus Carcinoma



Glial Tumors of Uncertain Origin
17 9430/3 Astroblastoma
18 9381/3 Gliomatosis Cerebri
19 9444/1 Chordoid Glioma of the Third Ventricle



Neuronal and Mixed Neuronal-Glial Tumors
20 9492/0 Gangliocytoma
21 9493/0 Dysplastic Gangliocytoma of Cerebellum (Lhermitte-Duclos)
22 9412/1 Desmoplastic Infantile Astrocytoma / Ganglioglioma
23 9413/0 Dysembryoplastic Neuroepithelial Tumor
24 9505/1 Ganglioglioma
25 9505/3 Anaplastic Ganglioglioma
26 9506/1 Central Neurocytoma
27 9506/1 Cerebellar Liponeurocytoma
28 8680/1 Paraganglioma of the Filum Terminale



Neuroblastic Tumors
29 9522/3 Olfactory Neuroblastoma (Aaesthesioneuroblastoma)
30 9523/3 Olfactory Neuroepithelioma
31 9500/3 Neuroblastomas of the Adrenal Gland ans Sympathetic Nervous System



Pineal Parenchymal Tumors
32 9361/1 Pineocytoma
33 9362/3 Pineoblastoma
34 9362/3 Pineal Parenchymal Tumor of Intermediate Differentiation



Embryonal Tumors
35 9501/3 Medulloepithelioma
36 9392/3 Ependymoblastoma
37 9470/3 Medulloblastoma
(Variant) 9471/3 Desmoplastic Medulloblastoma
(Variant) 9474/3 Large Cell Medulloblastoma
(Variant) 9472/3 Medullomyoblastoma
(Variant) 9470/3 Melanotic Medulloblastoma
38 9473/3 Supratentorial Primitive Neuroectodermal Tumor (PNET)
(Variant) 9500/3 Neuroblastoma
(Variant) 9490/3 Ganglioneuroblastoma
39 9508/3 Atypical Teratoid/Rhabdoid Tumor


Tumors of Peripheral Nerves

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40 9560/0 Schwannoma (Neurilemmoma, Neurinoma)
(Variant) 9560/0 Cellular
(Variant) 9560/0 Plexiform
(Variant) 9560/0 Melanotic



41 9540/0 Neurofibroma
(Variant) 9550/0 Plexiform



42 9571/0 Perineurioma
(Variant) 9571/0 Intraneural Perineurioma
(Variant) 9571/0 Soft Tissue Perineurioma



43 9540/3 Malignant Peripheral Nerve Sheath Tumor (MPNST)
(Variant) 9540/3 Epithelioid
(Variant) 9540/3 MPNST with Divergent Mesenchymal and/or Epithelial Differentiation
(Variant) 9540/3 Melanotic
(Variant) 9540/3 Melanotic Psammomatous


Tumors of the Meninges

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Tumors of Meningothelial Cells
44 9530/0 Meningioma
(Variant) 9531/0 Meningothelial
(Variant) 9532/0 Fibrous (Fibroblastic)
(Variant) 9537/0 Transitional (Mixed)
(Variant) 9533/0 Psammomatous
(Variant) 9534/0 Angiomatous
(Variant) 9530/0 Microcystic
(Variant) 9530/0 Secretory
(Variant) 9530/0 Lymphoplasmacyte-Rich
(Variant) 9530/0 Metaplastic
(Variant) 9538/1 Clear Cell
(Variant) 9538/1 Chordoid
(Variant) 9539/1 Atypical
(Variant) 9538/3 Papillary
(Variant) 9538/3 Rhabdoid
(Variant) 9530/3 Anaplastic Meningioma



Mesenchymal, Non-Meningothelial Tumors
45 8850/0 Lipoma
46 8861/0 Angiolipoma
47 8880/0 Hibernoma
48 8850/3 Liposarcoma (Intracranial)
49 8815/0 Solitary Fibrous Tumor
50 8810/3 Fibrosarcoma
51 8830/3 Malignant Fibrous Histiocytoma
52 8890/0 Leiomyoma
53 8890/3 Leiomyosarcoma
54 8900/0 Rhabdomyoma
55 8900/3 Rhabdomyosarcoma
56 9220/0 Chondroma
57 9220/3 Chondrosarcoma
58 9180/0 Osteoma
59 9180/3 Osteosarcoma
60 9210/0 Osteochondroma
61 9120/0 Haemangioma
62 9133/1 Epithelioid Haemangioendothelioma
63 9150/1 Haemangiopericytoma
64 9120/3 Angiosarcoma
65 9140/3 Kaposi Sarcoma



Primary Melanocytic Lesions
66 8728/0 Diffuse Melanocytosis
67 8728/1 Melanocytoma
68 8720/3 Malignant Melanoma
69 8728/3 Meningeal Melanomatosis



Tumors of Uncertain Histogenesis
70 9161/1 Haemangioblastoma


Lymphomas and Haemopoietic Neoplasms

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71 9590/3 Malignant Lymphomas
72 9731/3 Plasmacytoma
73 9930/3 Granulocytic Sarcoma


Germ Cell Tumors

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74 9064/3 Germinoma
75 9070/3 Embryonal Carcinoma
76 9071/3 Yolk Sac Tumor
77 9100/3 Choriocarcinoma
78 9080/1 Teratoma
(Variant) 9080/0 Mature
(Variant) 9080/3 Immature
(Variant) 9084/3 Teratoma with Malignant Transformation
79 9085/3 Mixed Germ Cell Tumors


Tumors of the Sellar Region

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80 9350/1 Craniopharyngioma
(Variant) 9351/1 Adamantinomatous
(Variant) 9352/1 Papillary
81 9582/0 Granular Cell Tumor


Metastatic Tumors

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Notes

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(*) Morphology code of the International Classification of Diseases for Oncology (ICD-O) and the Systematized Nomenclature of Medicine (SNOMED). Behaviour is coded /0 for benign tumors, /1 for low or uncertain malignant potential or borderline malignancy, (/2 for in situ lesions) and /3 for malignant tumors.
(**) The italicised numbers are provisional codes proposed for the third edition of ICD-O. They should, for the most part, be incorporated into the next edition of ICD-O, but they are subject to change.


Updates

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Brat DJ, Scheithauer BW, Fuller GN, Tihan T.
Newly Codified Glial Neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: Angiocentric Glioma, Pilomyxoid Astrocytoma and Pituicytoma.
Brain Pathol. 2007 Jul;17(3):319-24.
  Abstract |   Reprint |   Related articles

Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P.
The 2007 WHO Classification of Tumours of the Central Nervous System.
Acta Neuropathol (Berl). 2007 Aug;114(2):97-109. Epub 2007 Jul 6.
  Abstract |   Reprint |   Related articles

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