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Journal of Pediatric Neurology 2024; 22(03): 186-193
DOI: 10.1055/s-0043-1768029
Review Article

BRAF Landscape and Its Implications among Patients with Pediatric Low-Grade Gliomas: A Comprehensive Review of the Literature

Mohammed A. Fouda 
1   Department of Neurological Surgery, New York-Presbyterian Hospital, Weill Cornell Medicine, New York, New York, United States
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Abstract

Low-grade gliomas are the most common intracranial tumor in the pediatric population. Pediatric low-grade gliomas represent a heterogeneous group of tumors. Genetic alterations that result in upregulation of the MAPK/ERK pathway represent most of the genetic landscape of pediatric low-grade gliomas. BRAF-V600E mutant pediatric low-grade gliomas may represent a unique and aggressive subset of tumors that require targeted therapy especially if gross total resection is not feasible. Many patients with pediatric low-grade gliomas have demonstrated successful clinical and radiological responses to BRAF and/or MEK inhibitors. Given the high proportion of patients who fail to respond to the current standard chemotherapy and radiotherapy, these targeted therapies should be considered in future trials and further investigations. In this review of the literature, we summarize the molecular status of BRAF alterations among patients with pediatric low-grade gliomas and provide an update on previous and current BRAF and MEK inhibitors clinical trials.

Keywords

pediatric - low-grade glioma - BRAF - BRAF inhibitors - MEK inhibitors


Publication History

Received: 07 February 2023

Accepted: 02 March 2023

Article published online:
12 April 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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