WHO CLASSIFICATION OF THE TUMORS OF THE CNS 2016
  1. Diffuse astrocytic and oligodendroglial tumours

    1. Diffuse astrocytoma, IDH – mutant 9400/3
      - Gemistocytic astrocytoma, IDH – mutant 9411/3

    2. Diffuse astrocytoma, IDH – wildtype 9400/3

    3. Diffuse astrocytoma, NOS 9400/3

    4. Anaplastic astrocytoma, IDH – mutant 9401/3

    5. Anaplastic astrocytoma, IDH – wildtype 9401/3

    6. Anaplastic astrocytoma, NOS 9401/3

    7. Glioblastoma, IDH – wildtype 9440/3
      - Giant cell glioblastoma 9441/3
      - Gliosarcoma 9442/3
      - Epithelioid glioblastoma 9440/3

    8. Glioblastoma, IDH – mutant 9445/3

    9. Glioblastoma, NOS 9440/3

    10. Diffuse midline glioma, H3 K27M – mutant 9385/3

    11. Oligodendroglioma, IDH – mutant and 1p/19q-codeleted 9450/3

    12. Oligodendroglioma, NOS 9450/3

    13. Anaplastic oligodendroglioma, IDH – mutant and 1p/19q-codeleted 9451/3

    14. Anaplastic oligodendroglioma, NOS 9451/3

    15. Oligoastrocytoma, NOS 9382/3

    16. Anaplastic oligoastrocytoma, NOS 9451/3

  2. Other astrocytic tumours

    1. Pilocytic astrocytoma 9421/1
      - Pilomyxoid astrocytoma 9425/3

    2. Sub ependymal giant cell astrocytoma 9384/1

    3. Pleomorphic xanthoastrocytoma 9424/3

    4. Anaplastic pleomorphic xanthoastrocytoma 9424/3

  3. Ependymal tumours

    1. Subependymoma 9383/1

    2. Myxopapillary ependymoma 9394/1

    3. Ependymoma 9391/3
      - Papillary ependymoma 9393/3
      - Clear cell ependymoma 9391/3
      - Tanycytic ependymoma 9391/3

    4. Ependymoma, RELA fusion – positive 9396/3

    5. Anaplastic ependymoma 9392/3

  4. Other gliomas

    1. Chordoid glioma of the third ventricle 9444/1

    2. Angiocentric glioma 9431/1

    3. Astroblastoma 9430/3

  5. Choroid plexus tumours

    1. Choroid plexus papilloma 9390/0

    2. Atypical choroid plexus papilloma 9390/1

    3. Choroid plexus carcinoma 9390/3

  6. Neuronal and mixed neuronal – glial tumours

    1. Dysembryoplastic neuroepithelial tumour 9413/0

    2. Gangliocytoma 9492/0

    3. Ganglioglioma 9505/1

    4. Anaplastic ganglioglioma 9505/3

    5. Dysplastic cerebellar gangliocytoma (Lhermitte – Duclos disease) 9493/0

    6. Desmoplastic infantile astrocytoma and ganglioglioma 9412/1

    7. Papillary glioneuronal tumour 9509/1

    8. Rosette – forming glioneuronal tumour 9509/1

    9. Diffuse leptomeningeal glioneuronal tumour

    10. Central neurocytoma 9506/1

    11. Extraventricular neurocytoma 9506/1

    12. Cerebellar liponeurocytoma 9506/1

    13. Paraganglioma 8693/1

  7. Tumours of the pineal region

    1. Pineocytoma 9361/1

    2. Pineal parenchymal tumour of intermediate differentiation 9362/3

    3. Pineoblastoma 9362/3

    4. Papillary tumour of the pineal region 9395/3

  8. Embryonal tumours

    1. Medulloblastoma, NOS 9470/3

    2. Medulloblastomas, genetically defined
      - Medulloblastoma, WNT – activated 9475/3*
      - Medulloblastoma, SHH – activated and TP53 – mutant 9476/3
      - Medulloblastoma, SHH – activated and TP53 – wildtype 9471/3
      - Medulloblastoma, non – WNT/non – SHH 9477/3*
      + Medulloblastoma, group 3
      + Medulloblastoma, group 4

    3. Medulloblastomas, histologically defined
      - Medulloblastoma, classic 9470/3
      - Desmoplastic/nodular medulloblastoma 9471/3
      - Medulloblastoma with extensive nodularity 9471/3
      - Large cell/anaplastic medulloblastoma 9474/3

    4. Embryonal tumour with multilayered rosettes, C19MC – altered 9478/3

    5. Embryonal tumour with multilayered rosettes, NOS 9478/3

    6. Other CNS embryonal tumours
      - Medulloepithelioma 9501/3
      - CNS neuroblastoma 9500/3
      - CNS ganglioneuroblastoma 9490/3
      - CNS embryonal tumour, NOS 9473/3

    7. Atypical teratoid/rhabdoid tumour 9508/3

    8. CNS embryonal tumour with rhabdoid features 9508/3

  9. Tumours of the cranial and paraspinal nerves

    1. Schwannoma 9560/0
      - Cellular schwannoma 9560/0
      - Plexiform schwannoma 9560/0

    2. Melanotic schwannoma 9560/1

    3. Neurofibroma 9540/0
      - Atypical neurofibroma 9540/0
      - Plexiform neurofibroma 9550/0

    4. Perineurioma 9571/0

    5. Hybrid nerve sheath tumours

    6. Malignant peripheral nerve sheath tumour (MPNST) 9540/3
      - MPNST with divergent differentiation
      - Epithelioid MPNST 9540/3
      - MPNST with perineurial differentiation 9540/3

  10. Meningiomas

    1. Meningioma 9530/0

    2. Maningioma variants
      - Meningothelial meningioma 9531/0
      - Fibrous meningioma 9532/0
      - Transitional meningioma 9537/0
      - Psammomatous meningioma 9533/0
      - Angiomatous meningioma 9534/0
      - Microcystic meningioma 9530/0
      - Secretory meningioma 9530/0
      - Lymphoplasmacyte – rich meningioma 9530/0
      - Metaplastic meningioma 9530/0
      - Chordoid meningioma 9538/1
      - Clear cell meningioma 9538/1
      - Atypical meningioma 9539/1
      - Papillary meningioma 9538/3
      - Rhabdoid meningioma 9538/3
      - Anaplastic (malignant) meningioma 9530/3

  11. Mesenchymal, non-meningothelial tumours

    1. Solitary fibrous tumour/haemangiopericytoma
      - Grade 1 8815/0
      - Grade 2 8815/1
      - Grade 3 8815/3

    2. Haemangioblastoma 9161/1

    3. Haemangioma 9120/0

    4. Epithelioid haemangioendothelioma 9133/3

    5. Angiosarcoma 9120/3

    6. Kaposi sarcoma 9140/3

    7. Ewing sarcoma/peripheral primitive neuroectodermal tumour 9364/3

    8. Lipoma 8850/0

    9. Angiolipoma 8861/0

    10. Hibernoma 8880/0

    11. Liposarcoma 8850/3

    12. Desmoid – type fibromatosis 8821/1

    13. Myofibroblastoma 8825/0

    14. Inflammatory myofibroblastic tumour 8825/1

    15. Benign fibrous histiocytoma 8830/0

    16. Fibrosarcoma 8810/3

    17. Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma 8802/3

    18. Leiomyoma 8890/0

    19. Leiomyosarcoma 8890/3

    20. Rhabdomyoma 8900/0

    21. Rhabdomyosarcoma 8900/3

    22. Chondroma 9220/0

    23. Chondrosarcoma 9220/3

    24. Osteoma 9180/0

    25. Osteochondroma 9210/0

    26. Osteosarcoma 9180/3

  12. Melanocytic tumours

    1. Meningeal melanocytosis 8728/0

    2. Meningeal melanomatosis 8728/3

    3. Meningeal melanocytoma 8728/1

    4. Meningeal melanoma 8728/3

  13. Lymphomas

    1. Diffuse large B – cell lymphoma of the CNS 9680/3
      - Corticoid – mitigated lymphoma
      - Sentinel lesions

    2. Immunodeficiency – associated CNS lymphomas
      - AIDS – related diffuse large B – cell lymphoma
      - EBV+ diffuse large B – cell lymphoma, NOS
      - Lymphomatoid granulomatosis 9766/1

    3. Intravascular large B – cell lymphoma 9712/3

    4. Miscellaneous rare lymphomas in the CNS
      - Low – grade B – cell lymphomas of the CNS
      - T – cel and NK/T – cell lymphomas of the CNS
      - Anaplastic large cell lymphoma, ALK – positive 9714/3
      - Anaplastic large cell lymphoma, ALK – negative 9702/3

    5. MALT lymphoma of the dura 9699/3

  14. Histiocytic tumours

    1. Langerhans cell histiocytosis 9751/3

    2. Erdheim – Chester disease 9750/1

    3. Rosai – Dorfman disease

    4. Juvenile xanthogranuloma

    5. Histiocytic sarcoma 9755/3

  15. Germ cell tumours

    1. Germinoma 9064/3

    2. Embryonal carcinoma 9070/3

    3. Yolk sac tumour 9071/3

    4. Choriocarcinoma 9100/3

    5. Teratoma 9080/1
      - Mature teratoma 9080/0
      - Immature teratoma 9080/3

    6. Teratoma with malignant transformation 9084/3

    7. Mixed germ cell tumour 9085/3

  16. Familial tumour syndromes

    1. Neurofi bromatosis type 1

    2. Neurofi bromatosis type 2

    3. Schwannomatosis

    4. Von Hippel – Lindau disease

    5. Tuberous sclerosis

    6. Li – Fraumeni syndrome

    7. Cowden syndrome

    8. Turcot syndrome
      - Mismatch repair cancer syndrome
      - Familial adenomatous polyposis

    9. Naevoid basal cell carcinoma syndrome

    10. Rhabdoid tumour predisposition syndrome

  17. Tumours of the sellar region

    1. Craniopharyngioma 9350/1
      - Adamantinomatous craniopharyngioma 9351/1
      - Papillary craniopharyngioma 9352/1

    2. Granular cell tumour of the sellar region 9582/0

    3. Pituicytoma 9432/1

    4. Spindle cell oncocytoma 8290/0

  18. Metastatic tumours
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