Table 1.
Summary of molecular landscape in pediatric gliomas.
| Molecular Alteration | Function | Tumor Type | Potential Biomarker |
|---|---|---|---|
| pLGG (RAS/MAPK pathway) | |||
| KIAA1549-BRAF fusion | Activation of BRAF kinase domain Deregulation of the RAS/MAPK pathway |
PA | Diagnostic marker Poor prognostic marker |
| BRAF V600E mutation | PA/PXA/GG/DA | ||
| FGFR1 | Upregulation of the RAS/MAPK pathway | CNS tumors | NA |
| NF-1 | Negative regulator of RAS | PA/DA | NA |
| pLGG (non-RAS/MAPK pathway) | |||
| MYB and MYBL1 | Control of proliferation and differentiation of hematopoietic and other progenitor cells | DA | NA |
| CDKN2A homozygous deletion | Non-coding of the gene for tumor suppressors, protein p14ARF and p16INK4A | PXA | Poor prognostic marker |
| pHGG | |||
| H3K27M mutation | Decrease levels of lysine 27 methylation | GBM/DIPG | Diagnostic marker Poor prognostic marker |
| H3G34R/V mutation | Changes the distribution of lysine 36 methylation | ||
| Infantile glioma | |||
| NTRK fusions | Upregulation of the RAS/MAPK and PI3K/AKT/mTOR pathways | Hemispheric HGG | Intermediate prognostic marker |
| ALK fusions | |||
| ROS1 fusions | |||
pLGG, pediatric low-grade glioma; MAPK, mitogen-activated protein kinase; PA, pilocytic astrocytoma; PXA, pleomorphic xanthoastrocytoma; GG, ganglioglioma; DA, diffuse astrocytoma; CNS, central nervous system; NA, not available; pHGG, pediatric high-grade glioma; GBM, glioblastoma; DIPG, diffuse intrinsic pontine glioma.