Advances in the management of pediatric low-grade glioma
4 affiliations
- 1Department of Pediatric Hematology/Oncology/BMT, CancerCare Manitoba, Winnipeg, Canada
- 2Department of Neurosciences, Division of Child Neurology, University of Montreal, CHU Sainte-Justine, Montreal, Canada
- 3Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada; Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, ON, Canada; Arthur and Sonia Labbatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada
- 4Department of Pediatric Hematology/Oncology/BMT, CancerCare Manitoba, Winnipeg, Canada; Paul Albrechtsen Institute, CancerCare Manitoba 675 McDermot Avenue, Winnipeg, Manitoba, R3E 0V9, Canada; Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Canada; Corresponding author.
Abstract
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.
Related
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- Comparative Genomic and Epigenetic Landscape of Histone H3.3 G34R and K27M-Mutated Pediatric High-Grade Glioma Suggests Divergent Precision Oncology Strategies
- Cytotoxic activity of selinexor in BRAFV600E-driven pediatric low-grade glioma 3D cultures.
BibTeX
@article{brizini2026advances,
title = {{Advances in the management of pediatric low-grade glioma}},
author = {Meziane Brizini and Sébastien Perreault and Julie Bennett and Hadeel Hassan},
journal = {Neurotherapeutics},
year = {2026},
month = oct,
doi = {10.1016/j.neurot.2026.e00952},
url = {https://doi.org/10.1016/j.neurot.2026.e00952},
note = {CC BY},
}
RIS
TY - JOUR
TI - Advances in the management of pediatric low-grade glioma
AU - Meziane Brizini
AU - Sébastien Perreault
AU - Julie Bennett
AU - Hadeel Hassan
PY - 2026
DA - 2026/10/24
JO - Neurotherapeutics
DO - 10.1016/j.neurot.2026.e00952
UR - https://doi.org/10.1016/j.neurot.2026.e00952
AB - 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.
SN - 1878-7479
SN - 1933-7213
ER -