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The Changing Landscape of Pediatric Low-Grade Gliomas: Clinical Challenges and Emerging Therapies
Ana S Guerreiro Stucklin1, Uri Tabori1, Michael A Grotzer2
1Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Pediatric low-grade gliomas (PLGGs) are common childhood brain tumors. Novel therapies targeting molecular pathways like Ras/MAPK are needed due to treatment challenges and risk of neurological sequelae.
Area of Science:
- Pediatric neuro-oncology
- Cancer biology
- Molecular targeted therapy
Background:
- Pediatric low-grade gliomas (PLGGs) are the most common pediatric brain tumors.
- While often histologically benign, unresectable PLGGs, particularly in young children with midline tumors, can lead to disease progression and long-term neurological deficits.
- Genetic predisposition syndromes, such as neurofibromatosis type 1 and tuberous sclerosis, influence PLGG behavior and treatment.
Purpose of the Study:
- To review the current management strategies for pediatric low-grade gliomas.
- To discuss the evolving landscape of PLGG treatment driven by molecular insights.
- To highlight the need for novel therapeutic approaches for challenging PLGG cases.
Main Methods:
- Review of current literature on pediatric low-grade glioma management.
- Analysis of the role of genetic predisposition syndromes in PLGG.
- Discussion of emerging molecular targets, focusing on the Ras/MAPK pathway.
Main Results:
- Current treatments for PLGG often lack sustained tumor regression, necessitating multiple lines of therapy.
- Understanding genetic syndromes associated with PLGG is crucial for tailored management.
- Alterations in the Ras/MAPK pathway represent promising targets for new PLGG therapies.
Conclusions:
- Optimal PLGG management requires multidisciplinary care addressing tumor control and treatment side effects.
- Novel molecular targets, especially within the Ras/MAPK pathway, are revolutionizing the approach to pediatric low-grade gliomas.
- There is a significant unmet need for more effective and less toxic therapies for pediatric low-grade gliomas.
Abstract:
Pediatric low-grade gliomas (PLGGs) are the most common brain tumors in children. Though histologically benign and associated with excellent outcome, patients with unresectable lesions--mostly young children with midline tumors--experience multiple progressions and are at increased risk for long-term neurological sequelae. PLGGs in children with underlying genetic predisposition syndromes--especially neurofibromatosis type 1 and tuberous sclerosis--have a distinct natural history and biology with important treatment implications. Given the complexity of medical issues, optimal management requires a large network of health care providers; treatment decisions must address both tumor control and potential side effects of the therapy. Current treatment strategies often fail to induce sustained tumor regression and many children require several lines of therapy, highlighting the need for novel therapies. Here, we review the current management of PLGG and discuss how new molecular targets--in particular alterations of the Ras/MAPK pathway--are rapidly changing our approach to PLGG.
