Pediatric low-grade glioma: State-of-the-art and ongoing challenges

Area of Science:

• Pediatric Oncology
• Neuro-oncology
• Molecular Pathology

Background:

• Pediatric low-grade glioma (pLGG) is the most frequent central nervous system (CNS) tumor in children, accounting for 30%-40% of cases.
• While mortality is low, pLGG significantly impacts children's quality of life, necessitating a chronic disease management approach.
• Advances in understanding molecular drivers offer new therapeutic targets, but optimal use and long-term effects of inhibitors require further investigation.

Purpose of the Study:

• To provide a comprehensive overview of the current state-of-the-art in pediatric low-grade glioma (pLGG).
• To highlight key areas including epidemiology, molecular landscape, treatment, and outcomes.
• To introduce related manuscripts on preclinical models, clinical trials, and treatment resistance in pLGG.

Main Methods:

• Review of current literature and expert consensus from the International Pediatric Low-Grade Glioma Coalition (iPLGGc).
• Synthesis of data on pLGG epidemiology, histology, molecular drivers, and treatment paradigms.
• Discussion of survival, functional outcomes, imaging response, and unresolved challenges.

Main Results:

• pLGG is a chronic condition requiring focus on long-term functional outcomes and quality of life.
• Molecular insights are guiding targeted therapies, yet challenges in optimization and understanding long-term toxicities persist.
• The iPLGGc provides a framework for addressing critical issues in pLGG management.

Conclusions:

• A comprehensive understanding of pLGG, from molecular basis to clinical outcomes, is crucial for improving patient care.
• Ongoing research and collaborative efforts are essential to overcome challenges in treating pLGG, particularly regarding targeted therapies and resistance.
• This overview sets the stage for further detailed investigations into pLGG models, clinical trials, and resistance mechanisms.