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diffuse-midline-glioma
Diffuse Midline Glioma
Diffuse midline glioma (H3K27M+) is a WHO Grade 4 pediatric/young adult brain tumor defined by H3K27M mutation; DIPG, thalamic, and spinal cord locations; median OS 12-15 months; ONC201 (imipridone) FDA-approved for relapsed/refractory H3K27M+ DMG; no curative systemic therapy.
Entry Metadata
| Field | Value |
|---|---|
| ID | diffuse-midline-glioma |
| Name | Diffuse Midline Glioma |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Schwartzentruber J, Korshunov A, Liu XY, et al. Driver mutations in histone H3.3 and chromatin remodelling genes in paediatric glioblastoma. Nature. 2012;482(7384):226-231. · PubMed 22286061
- Khuong-Quang DA, Buczkowicz P, Rakopoulos P, et al. K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. Acta Neuropathol. 2012;124(3):439-447. · PubMed 22661320