Clinical Characterization of FGFR-Altered Pediatric Low-Grade Gliomas.

Pediatric low-grade gliomas (pLGG) are the predominant childhood central nervous system tumors. While BRAF alterations are known to drive the majority of pLGGs, a subgroup contains activating mutations or fusions involving FGFR1/2/3. FGFR is a receptor tyrosine kinase that plays a crucial role in cell growth, differentiation, and survival through interactions with fibroblast growth factors. Furthermore, FGFR has been shown to interact with the RAS/MAPK, PI3K/AKT, and JAK/STAT pathways. The current understanding of the biological behavior, clinical trajectory, and effectiveness of targeted inhibition in FGFR-altered gliomas is notably limited. We sought to define the epidemiologic characteristics, molecular features, radiographic and histologic characteristics, and clinical course of patients with FGFR-altered pLGGs. We also explored the therapeutic implications of these abnormalities in pediatric cases and highlight both current and emerging treatment strategies for pediatric patients with FGFR-altered cancers.