WHO classification of CNS tumours

The WHO classification of CNS tumours is the most widely accepted system for classifying CNS tumours and is based on the histological characteristics of the tumour. The latest revision was published in 2007.

Explanatory 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

• WHO grade I
  • pilocytic astrocytoma 9421/11 - WHO grade I
  • subependymal giant cell astrocytoma 9384/1 - WHO grade I
• WHO grade II
  • pilomyxoid astrocytoma 9425/3* - WHO grade II
  • 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
• WHO grade III
  • anaplastic astrocytoma 9401/3 - WHO grade III
• WHO grade IV
  • 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