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Table 1 Histological diagnosis and the common molecular events of WHO-recognized pLGG. RTK: receptor tyrosine kinase, SNV: single nucelotide variant

From: Pediatric low-grade glioma in the era of molecular diagnostics

Histological DiagnosisCommon Molecular Events
Glial Tumors
 Pilocytic AstrocytomaKIAA1549-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%)
 Subependymal Giant Cell AstrocytomaTSC1/2 SNV (85-95%)
 Diffuse AstrocytomaBRAF p.V600E (20-40%)
MYBL1 alteration (5-10%)
KIAA1549-BRAF (5-10%)
FGFR1 SNV (2-5%)
H3.3 p.K27M (2-5%)
IDH1 p.R132H (2-5%)
Other RTK SNV/Fusions (2-3%)
 Pleomorphic XanthoastrocytomaBRAF p.V600E (80-90%)
 OligodendrogliomaFGFR1-TKD duplication (10-20%)
FGFR1 SNV (10-20%)
BRAF p.V600E (5-10%)
FGFR1-TACC1 (3-5%)
IDH1 p.R132H (3-5%)
1p/19q co-deletion (3-5%)
Mixed Glioneuronal Tumors
 GangliogliomaBRAF p.V600E (40-50%)
KIAA1549-BRAF (10-15%)
 Desmoplastic Infantile Astrocytoma and GangliogliomaBRAF pV600E/D (40-60%)
FGFR1 SNV (5-10%)
KIAA1549-BRAF (2-5%)
 Dysembryoplastic Neuroepithelial TumorFGFR1-TKD duplication (20-30%)
FGFR1 SNV (20-30%)
FGFR1-TACC1 (10-15%)
Other RTK SNV/Fusions (5-10%)
BRAF p.V600E (5-10)
 Papillary Glioneuronal TumorSLC44A1-PRKCA (80-90%)
 Rosette-forming Glioneuronal TumorPIK3CA SNV (20-30%)
KIAA1549-BRAF (20-30%)
FGFR1 SNV (20-30%)
 Angiocentric GliomaMYB (80-90%)
 Chordoid Glioma of Third VentriclePRKCA SNV (80-90%)
 Polymorphous Low-Grade Neuroepithelial tumor of the Young (PLNTY)BRAF p.V600E (30-40%)
FGFR2/3 Fusions (30-40%)
 Multinodular and vacuolating neuronal tumor (MVNT)MAP2K1 SNV/Indel (50-60%)
BRAF p.V600E (5-10%)
Other BRAF SNV (5-10%)
FGFR2 Fusions (3-5%)