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 Diagnosis | Common Molecular Events |
|---|---|
| Glial Tumors | |
| Pilocytic Astrocytoma | 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%) |
| Subependymal Giant Cell Astrocytoma | TSC1/2 SNV (85-95%) |
| Diffuse Astrocytoma | BRAF 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 Xanthoastrocytoma | BRAF p.V600E (80-90%) |
| Oligodendroglioma | FGFR1-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 | |
| Ganglioglioma | BRAF p.V600E (40-50%) KIAA1549-BRAF (10-15%) |
| Desmoplastic Infantile Astrocytoma and Ganglioglioma | BRAF pV600E/D (40-60%) FGFR1 SNV (5-10%) KIAA1549-BRAF (2-5%) |
| Dysembryoplastic Neuroepithelial Tumor | FGFR1-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 Tumor | SLC44A1-PRKCA (80-90%) |
| Rosette-forming Glioneuronal Tumor | PIK3CA SNV (20-30%) KIAA1549-BRAF (20-30%) FGFR1 SNV (20-30%) |
| Angiocentric Glioma | MYB (80-90%) |
| Chordoid Glioma of Third Ventricle | PRKCA 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%) |
