Metastatic Low‐Grade Glioma Successfully Treated in a Pediatric Patient With BRAF A598_T599insI Mutation

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BACKGROUND: Pediatric low-grade gliomas are common brain tumors often driven by MAPK pathway alterations, including rare BRAF mutations. CASE: This case report describes the first use of treatment combining dabrafenib and trametinib in a 10-year-old boy with pleomorphic xanthoastrocytoma harboring a BRAF A598_T599insI mutation. Surgery and chemotherapy failed, leading to metastatic progression; yet targeted therapy has achieved a sustained clinical and radiological response, lasting more than 2 years. CONCLUSION: This case highlights the potential of RAF/MEK inhibitors in rare BRAF-mutated tumors and underscores the need for research to optimize treatment duration, manage side effects, and explore their role in non-canonical mutations.

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