# H3 K27M (diffuse midline glioma)

Source: https://onco.cc/terms/h3k27m/  
OnCo record `h3k27m` (Term). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

A single change in a histone protein that defines diffuse midline glioma, the childhood brain tumour with the fewest treatment options, and now the target of the first approved drug for it.

## Summary

Lysine-to-methionine mutation at position 27 of histone H3 (H3F3A or HIST1H3B) causes global loss of H3K27 trimethylation. Defines WHO grade 4 diffuse midline glioma (including DIPG), median survival ~11 months, radiotherapy the only proven therapy until dordaviprone (2025). GD2 is overexpressed, enabling CAR-T.

## Fields

- Kind: Term
- Last checked: 2026-09-06

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Diffuse_midline_glioma
- Wikipedia: https://en.wikipedia.org/wiki/Diffuse_midline_glioma

## Connected records

- cancers: [Diffuse midline glioma, H3 K27-altered (including DIPG)](https://onco.cc/cancers/dipg-dmg/), [Glioma & glioblastoma](https://onco.cc/cancers/glioblastoma/), [Paediatric high-grade glioma (excluding diffuse midline glioma)](https://onco.cc/cancers/paediatric-high-grade-glioma/), [Spinal cord tumours (intramedullary and intradural)](https://onco.cc/cancers/spinal-cord-tumours/)
- technologies: [CAR-T for glioma (IL13Rα2, GD2, EGFRvIII, multi-target)](https://onco.cc/technologies/glioma-car-t/), [DNA methylation profiling](https://onco.cc/technologies/methylation-profiling/)
- drugs: [Dordaviprone](https://onco.cc/drugs/dordaviprone/)
- people: [Crystal L. Mackall](https://onco.cc/people/crystal-mackall/)
- bottlenecks: [The brain: barrier and sanctuary](https://onco.cc/bottlenecks/b-brain-delivery/)
- ideas: [Develop drugs in children first when the target is a children's target](https://onco.cc/ideas/idea-bio2-paediatric-first-development/), [Read the spinal fluid to track brain tumours without opening the skull](https://onco.cc/ideas/idea-bio2-csf-ctdna-monitoring/)
- pathways: [Epigenetic reprogramming](https://onco.cc/pathways/epigenetic-reprogramming/)
- collections: [National Brain Tumor Society (NBTS)](https://onco.cc/collections/national-brain-tumor-society/)
- terms: [Ependymoma molecular groups (PF-A, PF-B, ZFTA, YAP1, MYCN)](https://onco.cc/terms/ependymoma-molecular-groups/), [Epigenetic progenitor theory: cancer without a first mutation](https://onco.cc/terms/epigenetic-progenitor-theory/)
- trials: [ACTION](https://onco.cc/trials/action-dmg/)
- targets: [Histone H3.3 (H3-3A)](https://onco.cc/targets/h3-3a/)
- biomarkers: [H3 K27M mutation](https://onco.cc/biomarkers/h3-k27m/)

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