WHO classification of tumours of the central nervous system

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MRI of a patient with anaplastic astrocytoma

The following is a simplified (deprecated) version of the last 2007 WHO classification of the tumours of the central nervous system.[1] [2] Currently, as of 2016, clinicians are using revised WHO grade 4th edition which incorporates recent advance in molecular pathology. The 4th revised edition, as the name implies, will soon be updated to catch up ever growing knowledge of this field.

Listed for each tumour are the WHO official name, the ICD-O code[3] (with Arabic numeral, where /0 indicates "benign" tumour, /3 malignant tumour and /1 borderline tumour), and with Roman numeral the WHO Grade (a parameter connected with the "aggressiveness" of the tumour).[note 1]

1. Tumours of neuroepithelial tissue[edit]

1.1. Astrocytic tumours (Astrocytomas, a type of brain tumor)
1.1.1 Pilocytic astrocytoma (ICD-O 9421/1, WHO grade I)
1.1.1a Pilomyxoid astrocytoma (ICD-O 9425/3, WHO grade II)
1.1.2 Subependymal giant cell astrocytoma (ICD-O 9384/1, WHO grade I)
1.1.3 Pleomorphic xanthoastrocytoma (ICD-O 9424/3, WHO grade II)
1.1.4 Diffuse astrocytoma (ICD-O 9400/3, WHO grade II)
1.1.5 Anaplastic astrocytoma (ICD-O 9401/3, WHO grade III)
1.1.6.Glioblastoma (ICD-O 9440/3, WHO grade IV)
1.1.6a Giant cell glioblastoma (ICD-O 9441/3, WHO grade IV)
1.1.6b Gliosarcoma (ICD-O 9442/3, WHO grade IV)
1.1.7 Gliomatosis cerebri (ICD-O 9381/3, WHO grade III)
1.2. Oligodendroglial tumours
1.2.1 Oligodendroglioma (ICD-O 9450/3, WHO grade II)
1.2.2 Anaplastic oligodendroglioma (ICD-O 9451/3, WHO grade III)
1.3. Oligoastrocytic tumours
1.3.1 Oligoastrocytoma (ICD-O 9382/3, WHO grade II)
1.3.2 Anaplastic oligoastrocytoma (ICD-O 9382/3, WHO grade III)
1.4. Ependymal tumours
1.4.1 Subependymoma (ICD-O 9383/1, WHO grade I)
1.4.2 Myxopapillary ependymoma (ICD-O 9394/1, WHO grade I)
1.4.3 Ependymoma (ICD-O 9391/3, WHO grade II)
1.4.4 Anaplastic ependymoma (ICD-O 9392/3, WHO grade III)
1.5. Choroid plexus tumours
1.5.1 Choroid plexus papilloma (ICD-O 9390/0, WHO grade I)
1.5.2 Atypical choroid plexus papilloma ( ICD-O 9390/1, WHO grade II)
1.5.3 Choroid plexus carcinoma (ICD-O 9390/3, WHO grade III)
1.6. Other neuroepithelial tumours
1.6.1 Astroblastoma (ICD-O 9430/3, WHO grade I)
1.6.2 Chordoid glioma of the third ventricle (ICD-O 9444/1, WHO grade II)
1.6.3 Angiocentric glioma (ICD-O 9431/1, WHO grade I)
1.7. Neuronal and mixed neuronal-glial tumours
1.7.1 Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos) (ICD-O 9493/0)
1.7.2 Desmoplastic infantile astrocytoma/ganglioglioma (ICD-O 9412/1, WHO grade I)
1.7.3 Dysembryoplastic neuroepithelial tumour (ICD-O 9413/0, WHO grade I)
1.7.4 Gangliocytoma (ICD-O 9492/0, WHO grade I)
1.7.5 Ganglioglioma (ICD-O 9505/1, WHO grade I)
1.7.6 Anaplastic ganglioglioma (ICD-O 9505/3, WHO grade III)
1.7.7 Central neurocytoma (ICD-O 9506/1, WHO grade II)
1.7.8 Extraventricular neurocytoma (ICD-O 9506/1, WHO grade II)
1.7.9 Cerebellar liponeurocytoma (ICD-O 9506/1, WHO grade II)
1.7.10 Papillary glioneuronal tumour (ICD-O 9509/1, WHO grade I)
1.7.11 Rosette-forming glioneuronal tumour of the fourth ventricle (ICD-O 9509/1, WHO grade I)
1.7.12 Paraganglioma (ICD-O 8680/1, WHO grade I)
1.8. Tumours of the pineal region
1.8.1 Pineocytoma (ICD-O 9361/1, WHO grade I)
1.8.2 Pineal parenchymal tumour of intermediate differentiation (ICD-O 9362/3, WHO grade II, III)
1.8.3 Pineoblastoma (ICD-O 9362/3, WHO grade IV)
1.8.4 Papillary tumors of the pineal region (ICD-O 9395/3, WHO grade II, III)
1.9. Embryonal tumours
1.9.1 Medulloblastoma (ICD-O 9470/3, WHO grade IV)
1.9.1b Medulloblastoma with extensive nodularity (ICD-O 9471/3, WHO grade IV)
1.9.1c Anaplastic medulloblastoma (ICD-O 9474/3, WHO grade IV)
1.9.2. CNS Primitive neuroectodermal tumour (ICD-O 9473/3, WHO grade IV)
1.9.2a CNS Neuroblastoma (ICD-O 9500/3, WHO grade IV)
1.9.3 Atypical teratoid/rhabdoid tumour (ICD-O 9508/3, WHO grade IV)

2. Tumours of cranial and paraspinal nerves[edit]

2.1 Schwannoma (ICD-O 9560/0, WHO grade I)
2.2 Neurofibroma (ICD-O 9540/0, WHO grade I)
2.3 Perineurioma (ICD-O 9571/0, 9571/3, WHO grade I, II, III)
2.4 Malignant peripheral nerve sheath tumour (MPNST) (ICD-O 9540/3, WHO grade II, III, IV)

3. Tumours of the meninges[edit]

3.1 Tumours of meningothelial cells
3.1.1 Meningioma (ICD-O 9530/0, WHO grade I)
3.1.1l Atypical meningioma (ICD-O 9539/1, WHO grade II)
3.1.1o Anaplastic meningioma (ICD-O 9530/3, WHO grade III)
3.2 Mesenchymal tumours
3.2.1 Lipoma (ICD-O 8850/0)
3.2.2 Angiolipoma (ICD-O 8861/0)
3.2.3 Hibernoma (ICD-O 8880/0)
3.2.4 Liposarcoma (ICD-O 8850/3)
3.2.5 Solitary fibrous tumour (ICD-O 8815/0)
3.2.6 Fibrosarcoma (ICD-O 8810/3)
3.2.7 Malignant fibrous histiocytoma (ICD-O 8830/3)
3.2.8 Leiomyoma (ICD-O 8890/0)
3.2.9 Leiomyosarcoma (ICD-O 8890/3)
3.2.10 Rhabdomyoma (ICD-O 8900/0)
3.2.11 Rhabdomyosarcoma (ICD-O 8900/3)
3.2.12 Chondroma (ICD-O 9220/0)
3.2.13 Chondrosarcoma (ICD-O 9220/3)
3.2.14 Osteoma (ICD-O 9180/0)
3.2.15 Osteosarcoma (ICD-O 9180/3)
3.2.16 Osteochondroma (ICD-O 9210/0)
3.2.17 Haemangioma (ICD-O 9120/0)
3.2.18 Epithelioid hemangioendothelioma (ICD-O 9133/1)
3.2.19 Haemangiopericytoma (ICD-O 9150/1, WHO grade II)
3.2.20 Anaplastic haemangiopericytoma (ICD-O 9150/3, WHO grade III)
3.2.21 Angiosarcoma (ICD-O 9120/3)
3.2.22 Kaposi Sarcoma (ICD-O 9140/3)
3.2.23 Ewing Sarcoma - PNET (ICD-O 9364/3)
3.3 Primary melanocytic lesions
3.3.1 Diffuse melanocytosis (ICD-O 8728/0)
3.3.2 Melanocytoma (ICD-O 8728/1)
3.3.3 Malignant melanoma (ICD-O 8720/3)
3.3.4 Meningeal melanomatosis (ICD-O 8728/3)
3.4 Other neoplasms related to the meninges
3.4.1 Haemangioblastoma (ICD-O 9161/1, WHO grade I)

4. Tumors of the haematopoietic system[edit]

4.1 Malignant Lymphomas (ICD-O 9590/3)
4.2 Plasmocytoma (ICD-O 9731/3)
4.3 Granulocytic sarcoma (ICD-O 9930/3)

5. Germ cell tumours[edit]

5.1 Germinoma (ICD-O 9064/3)
5.2 Embryonal carcinoma (ICD-O 9070/3)
5.3 Yolk sac tumour (ICD-O 9071/3)
5.4 Choriocarcinoma (ICD-O 9100/3)
5.5 Teratoma (ICD-O 9080/1)
5.6 Mixed germ cell tumours (ICD-O 9085/3)

6. Tumours of the sellar region[edit]

6.1 Craniopharyngioma (ICD-O 9350/1, WHO grade I)
6.2 Granular cell tumour (ICD-O 9582/0, WHO grade I)
6.3 Pituicytoma (ICD-O 9432/1, WHO grade I)
6.4 Spindle cell oncocytoma of the adenohypophysis (ICD-O 8991/0, WHO grade I)

7. Metastatic Tumours[edit]

Tumors that originate outside CNS and spread secondarily to CNS via Hematogenous route or by direct invasion from adjacent tissues. Most common primary tumour site

7.1 Lung
7.2 Breast
7.3 Melanoma
7.4 Renal
7.5 Colorectal

Reference : WHO pathology of tumours of central nervous system-2007


1979 WHO classification (1st edition)[edit]

Zülch, Histological typing of tumours of the central nervous system. World Health Organization, Geneva

1993 WHO classification (2nd edition)[edit]

reflected the advances brought about by the introduction of immunohistochemistry into diagnostic pathology

Kleihues P, Burger PC, Scheithauer BW (eds) (1993) Histological typing of tumours of the central nervous system. World Health Organization international histological classification of tumours. Springer, Heidelberg

2000 WHO classification (3rd edition)[edit]

edited by Kleihues and Cavenee

Kleihues P, Cavenee WK (eds) (2000) World Health Organization Classification of Tumours. Pathology and genetics of tumours of the nervous system. IARC Press

2007 WHO classification (4th edition)[edit]

This is the classification used until 2015. This version covered many techniques to diagnose the disease. Which was also included in the textbook of neurosurgery (Youman 6th edition).

2016 WHO classification (4th revised edition)[edit]

This version is lately released version. This is not 5th but 4th revised version foretelling possibility of releasing upgraded version in near future.

See also[edit]



  1. ^ Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (Aug 2007). "The 2007 WHO Classification of Tumours of the Central Nervous System". Acta Neuropathol. 114 (2): 97–109. doi:10.1007/s00401-007-0243-4. PMC 1929165. PMID 17618441.
  2. ^ Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) (2007). World Health Organization Classification of Tumours of the Central Nervous System. IARC, Lyon ISBN 92-832-2430-2.
    This is the last (2007) WHO classification. On the Internet at the URL http://www.brainlife.org/classification.htm Archived 2011-01-13 at the Wayback Machine are the previous WHO classifications. Accessed on 2010-12-31.
  3. ^ ICD-0-3 SEER SITE/HISTOLOGY VALIDATION LIST Archived 2011-06-13 at the Wayback Machine. Accessed on 2009-07-12.

External links[edit]