WHO classification of adult-type and pediatric-type diffuse gliomas

Tumor type	CNS WHO grade	Characteristic molecular genetic alterations
Adult-type diffuse gliomas
Astrocytoma, IDH-mutant	2, 3, 4	IDH1, IDH2
Oligodendroglioma, IDH-mutant, 1p/19q-codeleted	2, 3	IDH1, IDH2, 1p/19q
Glioblastoma, IDH-wildtype	4	IDH-wildtype, chromosome 7 and 10, TERT, EGFR, others
Pediatric-type diffuse low-grade gliomas
Diffuse astrocytoma, MYB- or MYBL1-altered	1	MYB, MYBL1
Angiocentric glioma	1	MYB
Polymorphous low-grade neuroepithelial tumor of the young	1	BRAF, FGFR genes
Diffuse low-grade glioma, MAPK pathway-altered	NA*	MAPK pathway genes
Pediatric-type diffuse high-grade gliomas
Diffuse midline glioma, H3 K27-altered	4	H3 K27, EGFR, EZHIP
Diffuse hemispheric glioma, H3 G34-mutant	4	H3 G34
Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype	4	IDH-wildtype, H3-wildtype, methylome, EGFR, PDGFRA, MYCN
Infant-type hemispheric glioma	NA^¶	RTK genes

WHO: World Health Organization; CNS: central nervous system; IDH: isocitrate dehydrogenase; TERT: telomerase reverse transcriptase; EGFR: epidermal growth factor receptor; MYB: MYB proto-oncogene, transcription factor; MYBL1: MYB proto-oncogene-like 1; FGFR: fibroblast growth factor receptor; MAPK: mitogen-activated protein kinase; EZHIP: EZH inhibitor protein; PDGFRA: platelet-derived growth factor receptor alpha; MYCN: MYCN proto-oncogene, bHLH transcription factor; NA: not assigned; RTK: receptor tyrosine kinase.
* Low grade.
¶ High grade.

Adapted with permission from: WHO Classification of Tumours Editorial Board. Central nervous system tumours [Internet]. Lyon (France): International Agency for Research on Cancer; 2021 [cited 2022 February 17]. (WHO classification of tumours series, 5th ed.; vol. 6). Available from: https://tumourclassification.iarc.who.int/chapters/45.

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WHO classification of adult-type and pediatric-type diffuse gliomas