WHO classification of tumours of the central nervous system

The following is a simplified (deprecated) version of the 2021 WHO classification of the tumours of the central nervous system.[1] Currently, as of 2021, clinicians are using the WHO grade 5th edition, which incorporates recent advances in molecular pathology.

MRI of a patient with anaplastic astrocytoma

Listed for each tumour are the WHO official name, the ICD-O code[2] (with Arabic numeral, where /0 indicates "benign" tumour, /3 malignant tumour and /1 borderline tumour), and the WHO Grade (a parameter connected with the "aggressiveness" of the tumour), also in Arabic numerals as per the updated 2021 guidelines.[note 1]

1. Gliomas, glioneuronal tumors, and neuronal tumours
1.1 Adult-type diffuse gliomas
1.1.1 Astrocytoma, IDH-mutant
1.1.2 Oligodendroglioma, IDH-mutant, and 1p/19q-codeleted
1.1.3 Glioblastoma, IDH-wildtype
1.2 Pediatric-type diffuse low-grade gliomas
1.2.1 Diffuse astrocytoma, MYB- or MYBL1-altered
1.2.2 Angiocentric glioma
1.2.3 Polymorphous low-grade neuroepithelial tumor of the young (PLNTY)
1.2.4 Diffuse low-grade glioma, MAPK pathway-altered
1.3 Pediatric-type diffuse high-grade gliomas
1.3.1 Diffuse midline glioma, H3 K27-altered
1.3.2 Diffuse hemispheric glioma, H3 G34-mutant
1.3.3 Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype
1.3.4 Infant-type hemispheric glioma
1.4 Circumscribed astrocytic gliomas
1.4.1 Pilocytic astrocytoma
1.4.2 High-grade astrocytoma with piloid features
1.4.3 Pleomorphic xanthoastrocytoma
1.4.4 Subependymal giant cell astrocytoma
1.4.5 Chordoid glioma
1.4.6 Astroblastoma, MN1-altered
1.5 Glioneuronal and neuronal tumours
1.5.1 Ganglioglioma
1.5.2 Desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma
1.5.3 Dysembryoplastic neuroepithelial tumor
1.5.4 Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters
1.5.5 Papillary glioneuronal tumor
1.5.6 Rosette-forming glioneuronal tumor
1.5.7 Myxoid glioneuronal tumor
1.5.8 Diffuse leptomeningeal glioneuronal tumor
1.5.9 Gangliocytoma
1.5.10 Multinodular and vacuolating neuronal tumor
1.5.11 Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease)
1.5.12 Central neurocytoma
1.5.13 Extraventricular neurocytoma
1.5.14 Cerebellar liponeurocytoma
1.6 Ependymal tumours
1.6.1 Supratentorial ependymoma
1.6.1.1 Supratentorial ependymoma, ZFTA fusion-positive
1.6.1.2 Supratentorial ependymoma, YAP1 fusion-positive
1.6.2 Posterior fossa ependymoma
1.6.2.1 Posterior fossa ependymoma, group PFA
1.6.2.2 Posterior fossa ependymoma, group PFB
1.6.3 Spinal ependymoma
1.6.3.1 Spinal ependymoma, MYCN-amplified
1.6.4 Myxopapillary ependymoma
1.6.5 Subependymoma

2. Choroid plexus tumours
2.1 Choroid plexus papilloma
2.2 Atypical choroid plexus papilloma
2.3 Choroid plexus carcinoma

3. Embryonal tumours
3.1 Medulloblastoma
3.2 Atypical teratoid/rhabdoid tumour
3.3 Cribiform neuroepithelial tumour
3.4 Embryonal tumour with multilayered rosettes
3.5 CNS neuroblastoma, FOXR2-activated
3.6 CNS tumor with BCOR internal tandem duplication

4. Pineal tumours
4.1 Pineocytoma
4.2 Pineal parenchymal tumour of intermediate differentiation
4.3 Pineoblastoma
4.4 Papillary tumor of the pineal region
4.5 Desmoplastic myxoid tumour of the pineal region, SMARCB1-mutant

5. Cranial and paraspinal nerve tumours
5.1 Schwannoma
5.2 Neurofibroma
5.3 Perineurioma
5.4 Hybrid nerve sheath tumour
5.5 Malignant melanotic nerve sheath tumour
5.6 Malignant peripheral nerve sheath tumour
5.7 Paraganglioma

6. Meningioma

7. Mesenchymal, non-meningothelial tumours
7.1 Soft tissue tumours
7.1.1 Fibroblastic and myofibroblastic tumours
7.1.1.1 Solitary fibrous tumour
7.1.2 Vascular tumours
7.1.2.1 Hemangiomas and vascular malformations
7.1.2.2 Hemangioblastoma
7.1.3 Skeletal muscle tumours
7.1.3.1 Rhabdomyosarcoma
7.1.4 Uncertain differentiation
7.1.4.1 Intracranial mesenchymal tumour, FET-CREB fusion-positive
7.1.4.2 CIC-rearranged sarcoma
7.1.4.3 Primary intracranial sarcoma, DICER1-mutant
7.1.4.4 Ewing sarcoma
7.2 Chondro-osseous tumours
7.2.1 Chondrogenic tumours
7.2.1.1 Mesenchymal chondrosarcoma
7.2.1.2 Chondrosarcoma
7.2.2 Notochordal tumours
7.2.2.1 Chordoma (including poorly differentiated chordoma)

8. Melanocytic tumours
8.1 Diffuse meningeal melanocytic neoplasms
8.1.1 Meningeal melanocytosis and meningeal melanomatosis
8.2 Circumscribed meningeal melanocytic neoplasms
8.2.1 Meningeal melanocytoma and meningeal melanoma

9. Hematolymphoid tumours
9.1 Lymphomas
9.1.1 CNS lymphomas
9.1.1.1 Primary diffuse large B-cell lymphoma of the CNS
9.1.1.2 Immunodeficiency-associated CNS lymphoma
9.1.1.3 Lymphomatoid granulomatosis
9.1.1.4 Intravascular large B-cell lymphoma
9.1.2 Miscellaneous rare lymphomas in the CNS
9.1.2.1 MALT lymphoma of the dura
9.1.2.2 Other low-grade B-cell lymphomas of the CNS
9.1.2.3 Anaplastic large cell lymphoma (ALK+/ALK−)
9.1.2.4 T-cell and NK/T-cell lymphomas
9.2 Histiocytic tumors
9.2.1 Erdheim-Chester disease
9.2.2 Rosai-Dorfman disease
9.2.3 Juvenile xanthogranuloma
9.2.4 Langerhans cell histiocytosis
9.2.5 Histiocytic sarcoma

10. Germ cell tumours
10.1 Mature teratoma
10.2 Immature teratoma
10.3 Teratoma with somatic-type malignancy
10.4 Germinoma
10.5 Embryonal carcinoma
10.6 Yolk sac tumor
10.7 Choriocarcinoma
10.8 Mixed germ cell tumor

11. Tumors of the sellar region
11.1 Adamantinomatous craniopharyngioma
11.2 Papillary craniopharyngioma
11.3 Pituicytoma, granular cell tumor of the sellar region, and spindle cell oncocytoma
11.4 Pituitary adenoma/PitNET
11.5 Pituitary blastoma

12. Metastases to the CNS
12.1 Metastases to the brain and spinal cord parenchyma
12.2 Metastases to the meninges

History

1979 WHO classification (1st edition)
Zülch, Histological typing of tumours of the central nervous system. World Health Organization, Geneva

1993 WHO classification (2nd edition)
reflected the advances brought about by the introduction of immunohistochemistry into diagnostic pathology

Kleihues P, Burger PC, Scheithauer BW (eds) (1993) Histological typing of tumours of the central nervous system. World Health Organization international histological classification of tumours. Springer, Heidelberg

2000 WHO classification (3rd edition)
edited by Kleihues and Cavenee

Kleihues P, Cavenee WK (eds) (2000) World Health Organization Classification of Tumours. Pathology and genetics of tumours of the nervous system. IARC Press

2007 WHO classification (4th edition)

This is the classification used until 2015. This version covered many techniques to diagnose the disease. Which was also included in the textbook of neurosurgery (Youman 6th edition).

2016 WHO classification (4th revised edition)

This is the current version and is a substantial revision of the 4th edition.[3] The reason it is not the 5th edition is to bring the CNS volume in line with other systems that were not up to 5th editions yet.

WHO classification (5th edition)

The 5th edition, which is likely to incorporate many of the proposed changes outlined by the cIMPACT-NOW (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy - Not Official WHO). Its publication date is not readily available and it is currently listed as a future title on the IARC website.[4]

WHO Classification of Tumours (Online Edition)

Since February 19, 2020, the WHO tumors classification has been accessible online as a subscription service, which includes the revised 4th edition.[5]

See also

Notes
  1. See the article Grading of the tumors of the central nervous system.

References
  1. Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister SM, Reifenberger G, Soffietti R, von Deimling A, Ellison DW (Aug 2021). "The 2021 WHO Classification of Tumors of the Central Nervous System: a summary". Neuro-Oncology. 23 (8): 1231–1251. doi:10.1093/neuonc/noab106. PMC 8328013. PMID 34185076.
  2. ICD-0-3 SEER SITE/HISTOLOGY VALIDATION LIST Archived 2011-06-13 at the Wayback Machine. Accessed on 2009-07-12.
  3. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, eds. (2016). WHO Classification of Tumours of the Central Nervous System (Revised 4th ed.). Lyon: International Agency for Research on Cancer. ISBN 9789283244929.
  4. "WHO Classification of Tumours – Future Titles". Archived from the original on 30 September 2018.
  5. "WHO Classification of Tumours Online". International Agency for Research on Cancer.
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