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OncoMatch/DIPG / Diffuse Midline Glioma/H3 K27M

DIPG / Diffuse Midline GliomaH3 K27M Clinical Trials

4 recruiting trials·Updated daily from ClinicalTrials.gov

H3 K27M mutations in H3-3A (or H3C2/H3C3) define diffuse midline glioma — H3 K27-altered, a WHO grade 4 entity encompassing DIPG — and occur in >70% of pontine tumors in children and young adults. Dordaviprone (Modeyso; formerly ONC201), a protease activator, received FDA accelerated approval in August 2025 for adult and pediatric patients aged ≥1 year with H3 K27M-mutant diffuse midline glioma with disease progression following prior therapy; ORR was 22% and median DOR was 10.3 months. Trials investigate dordaviprone combinations, radiation sensitizers, and novel H3 K27M-targeted immunotherapy approaches.

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