Appendix 1: WHO Classification

Appendix 1: WHO Classification and Grading of Tumours of the Central Nervous System

Diffuse Astrocytic and Oligodendroglial Tumours

  • Diffuse astrocytoma, IDH-mutant
    • Gemistocytic astrocytoma, IDH-mutant
  • Diffuse astrocytoma, IDH-wildtype
  • Diffuse astrocytoma, NOS
  • Anaplastic astrocytoma, IDH-mutant
  • Anaplastic astrocytoma, IDH-wildtype
  • Anaplastic astrocytoma, NOS
  • Glioblastoma, IDH-wildtype
    • Giant cell glioblastoma
    • Gliosarcoma
    • Epithelioid glioblastoma
  • Glioblastoma, IDH-mutant
  • Glioblastoma, NOS
  • Diffuse midline glioma, H3 K27M–mutant
  • Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  • Oligodendroglioma, NOS
  • Anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  • Anaplastic oligodendroglioma, NOS
  • Oligoastrocytoma, NOS
  • Anaplastic oligoastrocytoma, NOS
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Other Astrocytic Tumours

  • Pilocytic astrocytoma
    • Pilomyxoid astrocytoma
  • Subependymal giant cell astrocytoma
  • Pleomorphic xanthoastrocytoma
  • Anaplastic pleomorphic xanthoastrocytoma

Ependymal Tumours

  • Subependymoma
  • Myxopapillary ependymoma
  • Ependymoma
    • Papillary ependymoma
    • Clear cell ependymoma
    • Tanycytic ependymoma
  • Ependymoma, RelA fusion–positive
  • Anaplastic ependymoma

Other Gliomas

  • Chordoid glioma of the third ventricle
  • Angiocentric glioma
  • Astroblastoma

Choroid Plexus Tumours

  • Choroid plexus Papilloma
  • Atypical choroid plexus papilloma
  • Choroid plexus carcinoma

Neuronal and Mixed Neuronal–glial Tumours

  • Dysembryoplastic neuroepithelial tumour
  • Gangliocytoma
  • Ganglioglioma
  • Anaplastic ganglioglioma
  • Dysplastic cerebellar gangliocytoma
    • (Lhermitte–Duclos disease)
  • Desmoplastic infantile astrocytoma and ganglioglioma
  • Papillary glioneuronal tumour
  • Rosette-forming glioneuronal tumour
  • Diffuse leptomeningeal glioneuronal tumour
  • Central neurocytoma
  • Extraventricular neurocytoma
  • Cerebellar liponeurocytoma
  • Paraganglioma

Tumours of the Pineal Region

  • Pineocytoma
  • Pineal parenchymal tumour of intermediate differentiation
  • Pineoblastoma
  • Papillary tumour of the pineal region

Embryonal Tumours

  • Medulloblastoma
  • Medulloblastoma, NOS
  • Medulloblastomas, genetically defined
  • Medulloblastoma, WNT-activated
    • Medulloblastoma, SHH-activated and TP53-mutant
    • Medulloblastoma, SHH-activated and TP53-wildtype
    • Medulloblastoma, non-WNT/non-SHH
  • Medulloblastomas, histologically defined
    • Medulloblastoma, classic
    • Desmoplastic/nodular medulloblastoma
    • Medulloblastoma with extensive nodularity
    • Large cell / anaplastic medulloblastoma
  • Embryonal tumour with multilayered rosettes, C19MC-altered
  • Embryonal tumour with multilayered rosettes, NOS
  • Other CNS embryonal tumours
    • Medulloepithelioma
    • CNS neuroblastoma
    • CNS ganglioneuroblastoma
    • CNS embryonal tumour, NOS
  • Atypical teratoid/rhabdoid tumour
  • CNS embryonal tumour with rhabdoid features

Tumours of the Cranial and Paraspinal Nerves

  • Schwannoma
    • Cellular schwannoma
    • Plexiform schwannoma
  • Melanotic schwannoma
  • Neurofibroma
    • Atypical neurofibroma
    • Plexiform neurofibroma
  • Perineurioma
  • Hybrid nerve sheath tumours
  • Malignant peripheral nerve sheath tumour (MPNST)
    • MPNST with divergent differentiation
    • Epithelioid MPNST
    • MPNST with perineurial differentiation

Meningiomas

  • Meningioma
  • Meningioma variants
    • Meningothelial meningioma
    • Fibrous meningioma
    • Transitional meningioma
    • Psammomatous meningioma
    • Angiomatous meningioma
    • Microcystic meningioma
    • Secretory meningioma
    • Lymphoplasmacyte-rich meningioma
    • Metaplastic meningioma
    • Chordoid meningioma
    • Clear cell meningioma
    • Atypical meningioma
    • Papillary meningioma
    • Rhabdoid meningioma
    • Anaplastic (malignant) meningioma

Mesenchymal, Non-meningothelial Tumours

  • Solitary fibrous tumour / haemangiopericytoma
  • Haemangioblastoma
  • Haemangioma 25
  • Epithelioid haemangioendothelioma
  • Angiosarcoma
  • Kaposi sarcoma
  • Ewing sarcoma / peripheral primitive neuroectodermal tumour
  • Lipoma
  • Angiolipoma
  • Hibernoma
  • Liposarcoma
  • Desmoid-type fibromatosis
  • Myofibroblastoma
  • Inflammatory myofibroblastic tumour
  • Benign fibrous histiocytoma
  • Fibrosarcoma
  • Undifferentiated pleomorphic sarcoma / malignant fibrous histiocytoma
  • Leiomyoma
  • Leiomyosarcoma
  • Rhabdomyoma
  • Rhabdomyosarcoma
  • Chondroma
  • Chondrosarcoma
  • Osteoma
  • Osteochondroma
  • Osteosarcoma

Melanocytic Tumours

  • Meningeal melanocytosis
  • Meningeal melanomatosis
  • Meningeal melanocytoma
  • Meningeal melanoma

Lymphomas

  • Diffuse large B-cell lymphoma of the CNS
    • Corticoid-mitigated lymphoma
    • Sentinel lesions
  • Immunodeficiency-associated CNS lymphomas
    • AIDS-related diffuse large B-cell lymphoma
    • EBV+ diffuse large B-cell lymphoma, NOS
    • Lymphomatoid granulomatosis
  • Intravascular large B-cell lymphoma
  • Miscellaneous rare lymphomas in the CNS
    • Low-grade B-cell lymphomas
    • T-cell and NK/T-cell lymphomas
    • Anaplastic large cell lymphoma (ALK+/ALK–)
  • MALT lymphoma of the dura

Histiocytic Tumours

  • Langerhans cell histiocytosis
  • Erdheim–Chester disease
  • Rosai–Dorfman disease
  • Juvenile xanthogranuloma
  • Histiocytic sarcoma

Germ Cell Tumours

  • Germinoma
  • Embryonal carcinoma
  • Yolk sac tumour
  • Choriocarcinoma
  • Teratoma
    • Mature teratoma
    • Immature teratoma
  • Teratoma with malignant Transformation
  • Mixed germ cell tumour

Familial Tumour Syndromes

  • Neurofibromatosis type 1
  • Neurofibromatosis type 2
  • Schwannomatosis
  • Von Hippel–Lindau disease
  • Tuberous sclerosis
  • Li–Fraumeni syndrome
  • Cowden syndrome
  • Turcot syndrome
  • Naevoid basal cell carcinoma syndrome
  • Rhabdoid tumour predisposition syndrome

Tumours of the Sellar Region

  • Craniopharyngioma
    • Adamantinomatous craniopharyngioma
    • Papillary craniopharyngioma
  • Granular cell tumour of the sellar region
  • Pituicytoma
  • Spindle cell oncocytoma

Metastatic Tumours

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Last update: 18 September 2017