Special Feature: The 61st Annual Meeting of the Japanese Society of Pediatric Radiology: Collaboration and Progress
Pediatric brain tumor update: From diagnosis to treatment
2026 Volume 42 Issue 1 Pages 38-45
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2026 Volume 42 Issue 1 Pages 38-45
The diagnostic approach for central nervous system (CNS) tumors is undergoing a transformation, with the WHO Classification of CNS Tumours, 5th Edition (WHO CNS5), placing a strong emphasis on molecular genetic diagnosis. A clear separation has been established through recognition of the distinct molecular biological bases of pediatric and adult brain tumors. This review outlines the key changes in WHO CNS5, particularly the classification of pediatric-type diffuse gliomas (pLGG/pHGG) and the characteristic pediatric molecular alterations (e.g., H3, BRAF mutations). The redefinition of the highly aggressive diffuse midline glioma (DMG), H3 K27-altered, and the latest molecular-targeted therapies, such as BRAF inhibitors, ONC201 targeting H3K27M, and CAR-T cell therapy, are also discussed. Finally, the review emphasizes the crucial role of the diagnostic radiologist as a “navigator of precision medicine” through a radio-genomics approach and multidisciplinary collaboration in molecular diagnosis and treatment monitoring.
