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Clinical Characterization of FGFR-Altered Pediatric Low-Grade Gliomas
Benjamin Posorske1, Elizabeth S Borden2, Shea Gallus3
1School of Medicine-Arizona, A.T. Still University, Mesa, Arizona, USA.
Abstract:
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.
Insights
Pediatric low-grade gliomas (pLGG) with FGFR alterations are common brain tumors. This study defines their characteristics and explores targeted therapies for these FGFR-altered pediatric cancers.
Area of Science:
- Pediatric oncology
- Neuro-oncology
- Molecular biology
Background:
- Pediatric low-grade gliomas (pLGG) are the most common childhood central nervous system tumors.
- While BRAF alterations are frequent drivers, a subset of pLGGs harbor activating mutations or fusions in Fibroblast Growth Factor Receptor 1/2/3 (FGFR1/2/3).
- FGFR signaling is critical for cell growth and survival, interacting with key pathways like RAS/MAPK, PI3K/AKT, and JAK/STAT.
Purpose of the Study:
- To characterize the epidemiology, molecular, radiographic, and histologic features of FGFR-altered pLGGs.
- To define the clinical course and outcomes for patients with FGFR-altered pLGGs.
- To explore therapeutic implications and current/emerging treatment strategies for pediatric FGFR-altered gliomas.
Main Methods:
- Retrospective analysis of a cohort of pediatric patients with FGFR-altered pLGGs.
- Comprehensive review of epidemiologic, molecular, radiographic, and histologic data.
- Evaluation of clinical outcomes and treatment responses.
Main Results:
- Detailed description of the patient cohort with FGFR-altered pLGGs.
- Identification of specific molecular alterations and their correlation with tumor characteristics.
- Analysis of treatment efficacy and clinical trajectories in this subgroup.
Conclusions:
- FGFR alterations represent a distinct molecular subgroup within pediatric low-grade gliomas.
- Understanding these alterations is crucial for defining clinical behavior and therapeutic strategies.
- Targeted therapies hold promise for improving outcomes in pediatric patients with FGFR-altered gliomas.
