Advances in the management of pediatric low-grade glioma

Pediatric low-grade gliomas (pLGGs), the most common CNS tumors in children, are increasingly recognized as chronic diseases with prolonged courses and cumulative morbidity. Long-term survival is excellent. Management depends on tumor location and often requires repeated therapy, with risk of long-term functional and neurocognitive impairment. This review synthesizes recent advances in pLGG management, integrating molecular classification, systemic therapies, and emerging diagnostic and surveillance technologies. Treatment advances have been driven by improved molecular characterization, particularly the recognition that most tumors are driven by RAS/MAPK pathway alterations. Integrated histologic-molecular classification has enabled biologically driven risk stratification and adoption of targeted therapies (BRAF, MEK, RAF inhibitors), reshaping treatment for molecularly selected patients. Additionally, recognition of cancer predisposition syndromes and incorporation of germline testing have expanded the scope of clinical decision-making. Emerging technologies including cerebrospinal fluid based liquid biopsy testing, artificial intelligence enabled imaging, advanced metabolic and intraoperative imaging, and digital functional monitoring offer new opportunities to refine diagnosis, surveillance, and response assessments. Despite major progress, challenges remain, including uncertainty about optimal treatment duration and discontinuation, and the management of long-term toxicities from prolonged pathway inhibition. Global disparities in access to molecular diagnostics and targeted therapies, along with financial toxicity from prolonged treatment, hinder equitable precision care. Contemporary pLGG management requires integrating molecular biology, functional outcomes, and long-term surveillance within a multidisciplinary framework. Ongoing research on sequencing, survivorship, and access is essential to improve long-term outcomes.