WHO
WHO-classification of CNS-Tumours
A possibility of systematisation of cns-tumours is the distinction regarding the cellular origin, the cell composition and growth behavior, on which the classification of the World Health Organization (WHO) is based.
The latest version dates from 2007, which established - compared to previous versions - mostly a precise adjustment of the diagnosis and which is only in some cases a complete makeover. Particularly, three innovations were identified within the revision: new tumour entities, new variants of already defined entities and new differentiation patterns of known tumours without clinical or genetic characteristics.
According to the WHO, four different tumour grades are distinguished. This assessment is based on neuropathological, anatomical, histological and genetic characteristics of tumours. The evaluation of the tumour tissue under the microscope plays an important role.
WHO-grade I corresponds to a benign, slow-growing tumour. WHO-grade IV, in contrast, is typically a particularly virulent and fast-growing tumour. In the list below, all brain tumours are listed, which where defined by the WHO in 2007.
| Term of Brain Tumour | Grading | Code | |
| 1. | Diffuse astrocytic and oligodendroglial tumours | ||
| 1.1. | Diffuse astrocytoma, IDH-mutant | II | 9400/3 |
| 1.1.1 | • Gemistocytic astrocytoma, IDH-mutant | 9411/3 | |
| 1.2. | Diffuse astrocytoma, IDH-wildtype | 9400/3 | |
| 1.3. | Diffuse astrocytoma, NOS | 9400/3 | |
| 1.4. | Anaplastic astrocytoma, IDH-mutant | III | 9401/3 |
| 1.5. | Anaplastic astrocytoma, IDH-wildtype | 9401/3 | |
| 1.6. | Anaplastic astrocytoma, NOS | 9401/3 | |
| 1.7. | Glioblastoma, IDH-wildtype | IV | 9440/3 |
| 1.7.1. | • Giant cell glioblastoma | 9441/3 | |
| 1.7.2. | • Gliosarcoma | 9442/3 | |
| 1.7.3. | • Epitheloid glioblastoma | 9440/3 | |
| 1.8. | Glioblastoma, IDH-mutant | IV | 9445/3 |
| 1.9. | Glioblastoma, NOS | 9440/3 | |
| 1.10. | Diffuse midline glioma, H3 K27M-mutant | IV | 9385/3 |
| 1.11. | Oligodendroglioma, IDH-mutant and 1p/19q-codeleted | II | 9450/3 |
| 1.12. | Oligodendroglioma, NOS | 9405/3 | |
| 1.13. | Anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted | III | 9451/3 |
| 1.14. | Anaplastic oligodendroglioma, NOS | 9451/3 | |
| 1.15. | Oligoastrocytoma, NOS | 9382/3 | |
| 1.16. | Anaplastic oligoastrocytoma, NOS | 9382/3 | |
| 2. | Other astrocytic tumours | ||
| 2.1. | Pilocytic astrocytoma | I | 9421/1 |
| 2.1.1. | • Pilomyxoid astrocytoma | 9425/3 | |
| 2.2. | Subependymal giant cell astrocytoma | I | 9384/1 |
| 2.3. | Pleomorphic xanthoastrocytoma | II | 9424/3 |
| 2.4. | Anaplastic pleomorphic xanthoastrocytoma | III | 9424/3 |
| 3. | Ependymal tumours | ||
| 3.1. | Subependymoma | I | 9383/1 |
| 3.2. | Myxopapillary ependymoma | I | 9394/1 |
| 3.3. | Ependymoma | II | 9391/3 |
| 3.3.1. | • Papillary ependymoma | 9393/3 | |
| 3.3.2. | • Clear cell ependymoma | 9391/3 | |
| 3.3.3. | • Tanycytic ependymoma | 9391/3 | |
| 3.4. | Ependymoma, RELA fusion-positive | II/III | 9396/3 |
| 3.5. | Anaplastic ependymoma | III | 9392/3 |
| 4. | Other gliomas | ||
| 4.1. | Chordoid glioma of the 3rd ventricle | II | 9444/1 |
| 4.2. | Angiocentric glioma | I | 9431/1 |
| 4.3. | Astroblastoma | 9430/3 | |
| 5. | Choroid plexus tumours | ||
| 5.1. | Choroid plexus papilloma | I | 9390/0 |
| 5.2. | Atypical choroid plexus papilloma | II | 9390/1 |
| 5.3. | Choroid plexus carcinoma | III | 9390/3 |
| 6. | Neuronal and neuronal-glial tumours | ||
| 6.1. | Dysembryoplastic neuroepithelial tumour (DNT) | I | 9413/0 |
| 6.2. | Gangliocytoma | I | 9492/0 |
| 6.3. | Ganglioglioma | I | 9505/1 |
| 6.4. | Anaplastic ganglioglioma | III | 9505/3 |
| 6.5. | Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) | I | 9493/0 |
| 6.6. | Desmoplastic infantile astrocytoma/ganglioglioma | I | 9412/1 |
| 6.7. | Papillary glioneuronal tumour (PGNT) | I | 9509/1 |
| 6.8. | Rosette-forming glioneuronal tumour (RGNT) | I | 9509/1 |
| 6.9. | Diffuse leptomeningeal glioneuronal tumour | ||
| 6.10. | Central neurocytoma | II | 9506/1 |
| 6.11. | Extraventricular neurocytoma | II | 9506/1 |
| 6.12. | Cerebellar liponeurocytoma | II | 9506/1 |
| 6.13. | Paraganglioma | 8693/1 | |
| 7. | Tumours of the pineal region | ||
| 7.1. | Pineocytoma | I | 9361/1 |
| 7.2. | Pineal parenchymal tumour of intermediate differentation | II/III | 9362/3 |
| 7.3. | Pineoblastoma | IV | 9362/3 |
| 7.4. | Papillary tumour of the pineal region | II/III | 9395/3 |
| 8. | Embryonal tumours | ||
| 8.1. | Medulloblastomas, genetically defined | IV | |
| 8.1.1. | • Medulloblastoma, WNT-activated | IV | 9475/3 |
| 8.1.2. | • Medulloblastoma, WNT-activated and TP53-mutant | IV | 9476/3 |
| 8.1.3. | • Medulloblastoma, SHH-activated and TP53-wldtype | IV | 9471/3 |
| 8.1.4. | • Medulloblastoma, non-WNT/non-SHH | IV | 9477/3 |
| 8.1.4.1. | ⇒ Medulloblastoma, group 3 | ||
| 8.1.4.1. | ⇒ Medulloblastoma, group 4 | ||
| 8.2. | Medulloblastom, histologically defined | IV | |
| 8.2.1. | • medulloblastoma, classic | IV | 9470/3 |
| 8.2.2. | • medulloblastoma, desmoplastic/ nodular | IV | 9471/3 |
| 8.2.3. | • medulloblastoma with extensive nodularity | IV | 9471/3 |
| 8.2.4. | • medulloblastoma, large cell/anaplastic | IV | 9474/3 |
| 8.3. | Medulloblastoma, NOS | IV | 9470/3 |
| 8.4. | Embryonal tumour with multilayered rosettens, C19M-altered | IV | 9478/3 |
| 8.5. | Embryonal tumour with multilayered rosettes, NOS | 9478/3 | |
| 8.6. | Medulloepithelioma | IV | 9501/3 |
| 8.7. | CNS neuroblastoma | 9500/3 | |
| 8.9. | CNS ganglioneuroblastoma | 9490/3 | |
| 8.10. | CNS Embryonal tumour, NOS | IV | 9473/3 |
| 8.11. | Atypical teratoid/rhabdoid tumour (ATRT) | 9508/3 | |
| 8.12. | CNS Embryonal tumour with rhabdoid features | IV | 9508/3 |
| 9. | Tumours of the cranial and paraspinal nerves | ||
| 9.1. | Schwannoma | I | 9560/0 |
| 9.1.1. | • Cellulat schwannoma | 9560/0 | |
| 9.1.2. | • Plexiform schwannoma | 9560/0 | |
| 9.2. | Melanotic schwannoma | 9560/1 | |
| 9.3. | Neurofibroma | I | 9540/0 |
| 9.3.1. | • Atypical neurofibroma | 9540/0 | |
| 9.3.2. | • Plexiform neurofibroma | 9550/0 | |
| 9.4. | Perineurioma | I | 9571/0 |
| 9.5. | Hybrid nerve sheath tumour | ||
| 9.6. | Malignant peripheral nerve sheath tumour | II-IV | |
| 9.6.1. | • Epitheloid malignant peripheral nerve sheath tumour | 9540/3 | |
| 9.6.2. | • Malignant peripheral nerve sheath tumour with perineurial differentiation | 9540/3 | |
| 9540/3 | |||
| 10. | Meningeomas | ||
| 10.1. | Meningeoma | I | 9530/0 |
| 10.2. | Meningothelial meningeoma | 9531/0 | |
| 10.3. | Fibrous meningeoma | 9532/0 | |
| 10.4. | Transitional meningeoma | 9537/0 | |
| 10.5. | Psammomatous meningeoma | 9533/0 | |
| 10.6. | Angiomatous meningeoma | 9534/0 | |
| 10.7. | Microcystic meningeoma | 9530/0 | |
| 10.8. | Secretory meningeoma | 9530/0 | |
| 10.9. | Lymphoplasmacyte-rich meningeoma | 9530/0 | |
| 10.10 | Metaplastic meningeoma | 9530/0 | |
| 10.11. | Chordoid meningeoma | 9538/1 | |
| 10.12. | Clear cell meningeoma | 9538/1 | |
| 10.13. | Atypical meningeoma | II | 9539/1 |
| 10.14. | Papillary meningeoma | 9538/3 | |
| 10.15. | Rhabdoid meningeoma | 9538/3 | |
| 10.16. | Anaplastic meningeoma | III | 9530/3 |
| 11. | Mesenchymal, non-meningeothelial tumours | ||
| 11.1. | Solitary fibrous tumour/haemangiopericytoma | I-III | |
| 11.1.1. | • Grade I | 8815/0 | |
| 11.1.2. | • Grade II | 8815/1 | |
| 11.1.3. | • Grade III | 8815/3 | |
| 11.2. | Haemangioblastoma | I | 9161/1 |
| 11.3. | Haemangioma | 9120/0 | |
| 11.4. | Epitheloid Haemangioendothelioma | 9133/3 | |
| 11.5. | Angiosarcoma | 9120/3 | |
| 11.6. | Kaposi sarcoma | 9140/3 | |
| 11.7. | Ewing sarcoma/PNET | 9364/3 | |
| 11.8. | Lipoma | 8850/0 | |
| 11.9. | Angiolipoma | 8861/0 | |
| 11.10. | Hibernoma | 8880/0 | |
| 11.11. | Liposarcoma | 8850/3 | |
| 11.12. | Desmoid-type fibroma | 8821/1 | |
| 11.13. | Myofibroblastoma | 8825/0 | |
| 11.14. | Inflammatory myofibroblastic tumour | 8825/1 | |
| 11.15. | Benign fibrous histiocytoma | 8830/0 | |
| 11.16. | Fibrosarcoma | 8810/3 | |
| 11.17. | Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma | 8802/3 | |
| 11.18. | Leiomyoma | 8890/0 | |
| 11.19. | Leiomyosarcoma | 8890/3 | |
| 11.20. | Rhabdomyoma | 8900/0 | |
| 11.21. | Rhabdomyosarcoma | 8900/3 | |
| 11.22. | Chondroma | 9220/0 | |
| 11.23. | Chondrosarcoma | 9220/3 | |
| 11.24. | Osteoma | 9180/0 | |
| 11.25. | Osteochondroma | 9210/0 | |
| 11.26. | Osteosarcoma | 9180/3 | |
| 12. | Melanocytic tumours | ||
| 12.1. | Meningeal melanocytosis | 8728/0 | |
| 12.2. | Meningeal melanocytoma | 8728/1 | |
| 12.3. | Meningeal melanoma | 8720/3 | |
| 12.4. | Meningeal melanomatosis | 8728/3 | |
| 12.5. | |||
| 13. | Lymphomas | ||
| 13.1. | Diffuse large B-cell lymphoma of the CNS | 9680/3 | |
| 13.2. | Immunodeficiency-associated lymphoma of the CNS | ||
| 13.2.1. | • AIDS-related diffuse large B-cell lymphoma | ||
| 13.2.2. | • EBV-positive large B-cell lymphoma, NOS | ||
| 13.2.3. | • Lymphomatoid granulomatosis | 9766/1 | |
| 13.3. | Intravascular large B-cell lymphoma | 9712/3 | |
| 13.4. | Low-grade B-cell lymphoma of the CNS | ||
| 13.5. | T-cell and NK/T-cell lymphoma of the CNS | ||
| 13.6. | Anaplastic large cell lymphoma, ALK-positive | 9714/3 | |
| 13.7. | Anaplastic large cell lymphoma, ALK-negative | 9702/3 | |
| 13.8. | MALT lymphome of the dura | 9699/3 | |
| 14. | Histiocytic tumours | ||
| 14.1. | Langerhans cell histiocytosis | 9751/3 | |
| 14.2. | Erdheim-Chester disease | 9750/1 | |
| 14.3. | Rosai-Dorfman disease | ||
| 14.4. | Juvenile xanthogranuloma | ||
| 14.5. | Histiocytic sarcoma | 9755/3 | |
| 15. | Germ cell tumours | ||
| 15.1. | Germinoma | 9064/3 | |
| 15.2. | Embryonal carcinoma | 9070/3 | |
| 15.3. | Yolk sac tumour | 9071/3 | |
| 15.4. | Chorioncarcinoma | 9100/3 | |
| 15.5. | Teratoma | 9080/1 | |
| 15.5.1. | • Mature teratoma | 9080/0 | |
| 15.5.2. | • Immature teratoma | 9080/1 | |
| 15.6. | Teratoma with malignant transformation | 9084/3 | |
| 15.7. | Mixed germ cell tumour | 9085/3 | |
| 16. | Tumours of the sellar region | ||
| 16.1. | Craniopharyngeoma | I | 9650/1 |
| 16.1.1. | • Adamantinomatous craniopharyngeoma | 9351/1 | |
| 16.1.2. | • Papillary craniopharyngeoma | 9352/1 | |
| 16.2. | Granular cell tumour of the sellar region | I | 9582/0 |
| 16.3. | Pituicytoma | I | 9432/1 |
| 16.4. | Spindle cell oncocytoma (SCO) | I | 8290/0 |
| 17. | Metastatic tumours |
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