Table 2. Molecular profile and therapeutic modalities for the treatment of pediatric-type diffuse low-grade gliomas.
MYB: V-myb avian myeloblastosis viral oncogene homolog; MYBL1: V-myb avian myeloblastosis viral oncogene homolog-like 1; QKI: QKI, KH domain containing RNA binding); PCDHGA1: Protocadherin gamma subfamily A, 1; MMP16: Matrix metallopeptidase 16; MME: Membrane metalloendopeptidase; MAML2: Mastermind-like transcriptional coactivator 2; LOC105378099: an RNA gene, affiliated with the ncRNA class [56]; BRAFp.V600E: BRAF proto-oncogene, serine/threonine kinase, V600E refers to a specific mutation; FGFR: Fibroblast growth factor receptor; KIAA1549 (a protein-coding gene in Homo sapiens; SHTN1: Shootin 1; INA: Internexin neuronal intermediate filament protein alpha; TACC3: Transforming acidic coiled-coil containing protein 3; CTNNA3: Catenin alpha 3; FGFR(TKD)ITD: Fibroblast growth factor receptor tyrosine-kinase domain internal tandem duplications; NTRK: Neurotrophic tyrosine receptor kinase; MET: MET proto-oncogene, receptor tyrosine kinase; MAP2K1: Mitogen-activated protein kinase kinase 1; MEK: Mitogen-activated protein kinase kinase; mTOR: Mammalian target of rapamycin; AZD4547: An inhibitor of the FGFR family; LOC154902: Rare fusion partner of MYB gene in diffuse astrocytoma, MYB/MYBL1-altered [73].
| Tumor type | Molecular profile | Conventional treatment | Emerging therapies |
| Diffuse astrocytoma, MYB/MYBL1- Altered diffuse astrocytoma, MYB or MYBL1 altered | MYB/MYBL1 alterations (amplifications & gene fusions), fusion partners (QKI, PCDHGA1, MMP16, MME, MAML2, LOC105378099, LOC154902) | Gross total resection / maximum safe resection, focal radiotherapy, adjuvant conventional chemotherapy. | MEK Inhibitors (trametinib, selumetinib) (NCT03363217), mTOR inhibitors, MYB-targeted therapies [65-67] |
| Angiocentric glioma | MYB::QKI gene fusion [68-71] | Gross total resection / maximum safe resection. | MEK inhibitors (trametinib, selumetinib) (NCT03363217) |
| Polymorphous low-grade neuroepithelial tumor of the young | BRAFp.V600E & FGFR mutations (FGFR2, FGFR3), fusions (KIAA1549::BRAF, FGFR2: SHTN1, FGFR2: INA and FGFR3: TACC3, FGFR: CTNNA3) | Gross total resection, subtotal resection [19] | BRAF inhibitors (vemurafenib, dabrafenib) FGFR inhibitors (erdafitinib, AZD4547) [72-74] |
| Diffuse low-grade glioma, MAPK pathway-altered | BRAFp.V600E mutations, FGFR1 (TKD) duplications, FGFR1 hotspot mutations, NTRK1/2/3, MET, FGFR2 & MAP2K1 mutations. | Gross total resection, selective total resection. | BRAF inhibitors (trametinib, dabrafenib), MEK inhibitors (selumetinib), FGFR inhibitors (erdafitinib) |