[Recent Advances in the Classification and Treatment of Pediatric Gliomas].
This review summarizes WHO CNS5 molecular classification and genotype-directed treatment advances in pediatric gliomas, including MAPK-pathway targeting, dordaviprone for H3 K27-altered diffuse midline glioma, and fusion-directed therapies for infant-type hemispheric gliomas.
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This review summarizes WHO CNS5 molecular classification and genotype-directed treatment advances in pediatric gliomas, including MAPK-pathway targeting, dordaviprone for H3 K27-altered diffuse midline glioma, and fusion-directed therapies for infant-type hemispheric gliomas.
Research significance
The supplied review supports molecular profiling as a basis for selecting targeted therapies in pediatric glioma; it is reasonable to infer that matching MAPK alterations or NTRK, ROS1, and ALK fusions to corresponding inhibitors could improve outcomes in molecularly defined subgroups, but this record provides no primary efficacy, safety, or comparative outcome data.
Source abstract
Pediatric gliomas constitute the largest group of pediatric brain tumors and are biologically distinct from adult gliomas. The 2021 fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System (WHO CNS5) introduced a molecularly integrated classification system and recognized pediatric-type gliomas as distinct entities. In pediatric low-grade gliomas, activation of the MAPK pathway is a common molecular hallmark, with BRAF V600E mutations, BRAF fusions, FGFR1 alterations, and NF1-associated abnormalities representing major driver events. These discoveries have supported the development of targeted therapies, including dabrafenib plus trametinib, tovorafenib, and MEK inhibitors. In pediatric high-grade gliomas, diffuse midline glioma, H3 K27-altered, remains one of the most devastating tumors, although recent advances have led to the approval of dordaviprone in the United States. In addition, infant-type hemispheric gliomas frequently harbor NTRK, ROS1, or ALK fusions and may respond markedly to corresponding targeted therapies. Comprehensive molecular diagnostics have therefore become essential for accurate classification and treatment selection. This review summarizes the current WHO CNS5 classification of pediatric gliomas and highlights recent advances in molecularly guided therapeutic strategies.