Table 2.

Subclassification of pediatric-type diffuse midline gliomas, H3 K27-altered. Adapted from (4).

Diffuse midline glioma, H3.3 K27-mutant	H3.3 pK28M/I (K27M/I) mutation, often co-occurring with TP53/PPM1D mutation and PDGFRA alteration
Diffuse midline glioma, H3.1 or H3.2 K27-mutant	H3.1 or H3.2 pK28M (K27M) mutation, often co-occurring with PIK3CA, PIK3R1 or PTEN mutations, and ACVR1 mutation
Diffuse midline glioma, H3-wild-type with EZHIP overexpression	EZHIP overexpression
Diffuse midline glioma, EGFR- (and H3 K27-) mutant	EGFR mutation (insertion/deletion within exon 20 or p.A289T or p.A289V mutation), often co-occurring with TP53 mutation