WHO classification of the tumors of the central nervous system

Here it is presented a simplified version of the last WHO classification of the tumors of the central nervous system.^[1] ^[2]

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

1. Tumours of neuroepithelial tissue[]

1.1. Astrocytic tumours

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[]

2.1 Schwannoma (IDC-O 9560/0, WHO grade I)

2.2 Neurofibroma (IDC-O 9540/0, WHO grade I)

2.3 Perineurioma (IDC-O 9571/0, 9571/3, WHO grade I, II, III)

2.4 Malignant peripheral nerve sheath tumour (MPNST) (IDC-O 9540/3, WHO grade II, III, IV)

3. Tumours of the meninges[]

3.1 Tumours of meningothelial cells

3.1.1 Meningioma (IDC-O 9530/0, WHO grade I)

3.1.1l Atypical meningioma (IDC-O 9539/1, WHO grade II)

3.1.1o Anaplastic meningioma (IDC-O 9530/3, WHO grade III)

3.2 Mesenchymal tumours

3.2.1 Lipoma (IDC-O 8850/0)

3.2.2 Angiolipoma (IDC-O 8861/0)

3.2.3 Hibernoma (IDC-O 8880/0)

3.2.4 Liposarcoma (IDC-O 8850/3)

3.2.5 Solitary fibrous tumour (IDC-O 8815/0)

3.2.6 Fibrosarcoma (IDC-O 8810/3)

3.2.7 Malignant fibrous histiocytoma (IDC-O 8830/3)

3.2.8 Leiomyoma (IDC-O 8890/0)

3.2.9 Leiomyosarcoma (IDC-O 8890/3)

3.2.10 Rhabdomyoma (IDC-O 8900/0)

3.2.11 Rhabdomyosarcoma (IDC-O 8900/3)

3.2.12 Chondroma (IDC-O 9220/0)

3.2.13 Chondrosarcoma (IDC-O 9220/3)

3.2.14 Osteoma (IDC-O 9180/0)

3.2.15 Osteosarcoma (IDC-O 9180/3)

3.2.16 Osteochondroma (IDC-O 9210/0)

3.2.17 Haemangioma (IDC-O 9120/0)

3.2.18 Epithelioid hemangioendothelioma (IDC-O 9133/1)

3.2.19 Haemangiopericytoma (IDC-O 9150/1, WHO grade II)

3.2.20 Anaplastic haemangiopericytoma (IDC-O 9150/3, WHO grade III)

3.2.21 Angiosarcoma (IDC-O 9120/3)

3.2.22 Kaposi Sarcoma (IDC-O 9140/3)

3.2.23 Ewing Sarcoma - PNET (IDC-O 9364/3)

3.3 Primary melanocytic lesions

3.3.1 Diffuse melanocytosis (IDC-O 8728/0)

3.3.2 Melanocytoma (IDC-O 8728/1)

3.3.3 Malignant melanoma (IDC-O 8720/3)

3.3.4 Meningeal melanomatosis (IDC-O 8728/3)

3.4 Other neoplasms related to the meninges

3.4.1 Haemangioblastoma (IDC-O 9161/1, WHO grade I)

4. Tumors of the haematopoietic system[]

4.1 Malignant Lymphomas (IDC-O 9590/3)

4.2 Plasmocytoma (IDC-O 9731/3)

4.3 Granulocytic sarcoma (IDC-O 9930/3)

5. Germ cell tumours[]

5.1 Germinoma (IDC-O 9064/3)

5.2 Embryonal carcinoma (IDC-O 9070/3)

5.3 Yolk sac tumour (IDC-O 9071/3)

5.4 Choriocarcinoma (IDC-O 9100/3)

5.5 Teratoma (IDC-O 9080/1)

5.6 Mixed germ cell tumours (IDC-O 9085/3)

6. Tumours of the sellar region[]

6.1 Craniopharyngioma (IDC-O 9350/1, WHO grade I)

6.2 Granular cell tumour (IDC-O 9582/0, WHO grade I)

6.3 Pituicytoma (IDC-O 9432/1, WHO grade I)

6.4 Spindle cell oncocytoma of the adenohypophysis (IDC-O 8991/0, WHO grade I)

7. Metastatic Tumours[]

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Notes[]

↑ See the article Grading of the tumors of the central nervous system.

References[]

↑ Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007).
The 2007 WHO Classification of Tumours of the Central Nervous System
Acta Neuropathol 2007 Aug;114(2):97-109. Epub 2007 Jul 6.
↑ 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 are the previous WHO classifications. Accessed on 2010-12-31.
↑ ICD-0-3 SEER SITE/HISTOLOGY VALIDATION LIST. Accessed on 2009-07-12.

External links[]

AFIP Course Syllabus - Astrocytoma WHO Grading Lecture Handout

Template:Soft tissue tumors and sarcomas Template:Germ cell tumors

[4] See the article Grading of the tumors of the central nervous system.

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

[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 are the previous WHO classifications. Accessed on 2010-12-31.

[3] ICD-0-3 SEER SITE/HISTOLOGY VALIDATION LIST. Accessed on 2009-07-12.

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