. Author manuscript; available in PMC: 2024 Apr 1.

Published in final edited form as: Curr Neurol Neurosci Rep. 2023 Mar 7;23(4):185–199. doi: 10.1007/s11910-023-01257-3

Table 1.

WHO 2021 classification of pediatric low grade gliomas/glioneuronal tumors [1, 13, 54], tumor histologies and associated molecular alterations

WHO classification	Molecular alteration
Pediatric-type diffuse low-grade gliomas Diffuse astrocytoma, MYB- or MYBL1-altered Angiocentric glioma Polymorphous low-grade neuroepithelial tumor of the young Diffuse low-grade glioma, MAPK pathway-altered	MYB- or MYBL1-altered MYB-altered FGFR2/3 Fusions (30-40%) BRAF p.V600E (30-40%) -
Circumscribed astrocytic gliomas Pilocytic astrocytoma High-grade astrocytoma with piloid features Pleomorphic xanthoastrocytoma Subependymal giant cell astrocytoma Chordoid glioma Astroblastoma, MN1-altered	KIAA1549-BRAF (70-80%) FGFR1-TACC1 (3-5%) FGFR1 SNV (3-5%) BRAF p.V600E (3-5%) Other BRAF Fusions (2-5%) CRAF Fusions (2-5%) PTPN11 SNV (2-5%) KRAS/HRAS SNV (2-5%) BRAF p.V600E TSC1/2 SNV (85-95%) PRKCA SNV (80-90%) MN1
Glioneuronal and neuronal tumors Ganglioglioma Desmoplastic infantile ganglioglioma/astrocytoma Dysembryoplastic neuroepithelial tumor Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters Papillary glioneuronal tumor Rosette-forming glioneuronal tumor Myxoid glioneuronal tumor Diffuse leptomeningeal glioneuronal tumor Gangliocytoma Multinodular and vacuolating neuronal tumor Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) Central neurocytoma Extraventricular neurocytoma Cerebellar liponeurocytoma	BRAF p.V600E (40-50%) KIAA1549-BRAF (10-15%) BRAF pV600E/D (40-60%) FGFR1 SNV (5-10%) KIAA1549-BRAF (2-5%) FGFR1-TKD duplication (20-30%) FGFR1 SNV (20-30%) FGFR1-TACC1 (10-15%) Other RTK SNV/Fusions (5-10%) BRAF p.V600E (5-10) SLC44A1-PRKCA (80-90%) PIK3CA SNV (20-30%) KIAA1549-BRAF (20-30%) FGFR1 SNV (20-30%) MAP2K1 SNV/Indel (50-60%) BRAF p.V600E (5-10%) Other BRAF SNV (5-10%) FGFR2 Fusions (3-5%)