WHO classification of CNS tumours

The WHO classification of CNS tumours is the most widely accepted system for classifying CNS tumours. The latest revision was published in 2007.

Explainatory notes:

• the number after the slash refers to biological behaviour, not WHO Grade
• a * refers to a 'new' tumour in the classification

Also see the article on brain tumours and brain tumours in infancy for general discussion of these topics.

Tumours of neuroepithelial tissues

Astrocytic tumours

• pilocytic astrocytoma 9421/11 - WHO grade I
• pilomyxoid astrocytoma 9425/3* - WHO grade II
• subependymal giant cell astrocytoma 9384/1 - WHO grade I
• pleomorphic xanthoastrocytoma 9424/3 - WHO grade II
• diffuse astrocytoma 9400/3 - WHO grade II
• fibrillary astrocytoma 9420/3
• protoplasmic astrocytoma 9410/3
• gemistocytic astrocytoma 9411/3
• anaplastic astrocytoma 9401/3 - WHO grade III
• glioblastoma 9440/3 - WHO grade IV
• giant cell glioblastoma 9441/3 - WHO grade IV
• gliosarcoma 9442/3 - WHO grade IV
• gliomatosis cerebri 9381/3

Oligoastrocytic tumours

• oligoastrocytoma 9382/3 - WHO grade II
• anaplastic oligoastrocytoma 9382/3 - WHO grade III

Oligodendroglial tumours

• oligodendroglioma 9450/3 - WHO grade II
• anaplastic oligodendroglioma 9451/3 - WHO grade III

Ependymal tumours

• subependymoma 9383/1 - WHO grade I
• myxopapillary ependymoma 9394/1 - WHO grade I
• ependymoma 9391/3 - WHO grade II
• anaplastic ependymoma 9392/3 - WHO grade III

Choroid plexus tumours

• choroid plexus papilloma 9390/0 - WHO grade I
• atypical choroid plexus papilloma 9390/1* - WHO grade II
• choroid plexus carcinoma 9390/3 - WHO grade III

Other neuroepithelial tumours

• astroblastoma 9430/3
• chordoid glioma of the third ventricle 9444/1 - WHO grade II
• angiocentric glioma 9431/1* - WHO grade I

Neuronal and mixed neuronal-glial tumours

• dysplastic gangliocytoma of cerebellum - (Lhermitte-Duclos) - 9493/0 - WHO grade I
• desmoplastic infantile astrocytoma and ganglioglioma - 9412/1 - WHO grade I
• dysembryoplastic neuroepithelial tumour - 9413/0 - WHO grade I
• gangliocytoma - 9492/0 - WHO grade I
• ganglioglioma - 9505/1 - WHO grade I
• anaplastic ganglioglioma - 9505/3 - WHO grade III
• papillary glioneuronal tumor - 9509/1* - WHO grade I
• rosette-forming glioneuronal tumour of the fourth ventricle - 9509/1* - WHO grade ?
• central neurocytoma - 9506/1 - WHO grade II
• extraventricular neurocytoma - 9506/1* - WHO grade II
• cerebellar liponeurocytoma - 9506/1* - WHO grade II
• paraganglioma of the filum terminale - 8680/1 - WHO grade I

Tumours of the pineal region

• pineocytoma 9361/1 - WHO grade I
• pineal parenchymal tumour of intermediate differentiation 9362/3 - WHO grade II/III
• pineoblastoma 9362/3 - WHO grade IV
• papillary tumour of the pineal region 9395/3* - WHO grade II/III

Embryonal tumours

• medulloblastoma 9470/3 - WHO grade IV
• CNS primitive neuroectodermal tumour (PNET) 9473/3 - WHO grade IV
• CNS neuroblastoma 9500/3
• CNS ganglioneuroblastoma 9490/3
• medulloepithelioma 9501/3
• ependymoblastoma 9392/3
• atypical teratoid / rhabdoid tumour 9508/3 - WHO grade IV

Tumours of cranial and paraspinal nerves

• schwannoma (neurilemoma, neurinoma) 9560/0 - WHO grade I
• neurofibroma 9540/0 - WHO grade I
• perineurioma 9571/0 - WHO grade I / II / III
• malignant peripheral nerve sheath tumour (MPNST) 9540/3 - WHO grade II / III / IV

Tumours of the meninges

Tumours of meningothelial cells

• meningioma 9530/0 - WHO grade I
• atypical meningioma 9539/1 - WHO grade II
• anaplastic meningioma (malignant) 9530/3 - WHO grade III

Mesenchymal tumours

• lipoma 8850/0
• angiolipoma 8861/0
• hibernoma 8880/0
• liposarcoma (intracranial) 8850/3
• solitary fibrous tumour 8815/0
• fibrosarcoma 8810/3
• malignant fibrous histiocytoma 8830/3
• leiomyoma 8890/0
• leiomyosarcoma 8890/3
• rhabdomyoma 8900/0
• rhabdomyosarcoma 8900/3
• chondroma 9220/0
• chondrosarcoma 9220/3
• osteoma 9180/0
• osteosarcoma 9180/3
• osteochondroma 9210/0
• haemangioma 9120/0
• epithelioid haemangioendothelioma 9133/1
• haemangiopericytoma (CNS) 9150/1 - WHO grade II
• angiosarcoma 9120/3
• Kaposi sarcoma 9140/3

Primary melanocytic lesions

• diffuse melanocytosis 8728/0
• melanocytoma 8728/1
• malignant melanoma 8720/3
• meningeal melanomatosis 8728/3

Other neoplasms related to the meninges

• haemangioblastoma 9161/1 - WHO grade I

Lymphomas and haemopoietic neoplasma

• malignant lymphomas 9590/3
• plasmacytoma 9731/3
• granulocytic sarcoma 9930/3

Germ cell tumours

• germinoma (CNS) 9064/3
• embryonal carcinoma (CNS) 9070/3
• yolk sac tumour (CNS) 9071/3
• choriocarcinoma (CNS) 9100/3
• teratoma (CNS) 9080/1
• mixed germ cell tumours (CNS) 9085/3

see intracranial germ cell tumour article

Tumours of the sellar region

• craniopharyngioma 9350/1 - WHO grade I
• granular cell tumour 9582/0 - WHO grade I
• pituicytoma 9432/1* - WHO grade I
• spindle cell oncocytoma of the adenohypophysis 8291/0* - WHO grade I