The diffuse midline glioma H3 K27-altered occurs in midline structures such as the thalamus, pons, brainstem and spinal cord and is classified as a WHO Grade 4 tumor[^1][^2].
Epidemiology
The diffuse midline glioma H3 K27-altered primarily occurs in children, adolescents, and young adults [^1].
WHO Classification
Diffuse midline glioma H3 K27-altered was included in the WHO classification in 2016 as diffuse midline gliomas with H3-K27M mutation[^3]. In the WHO classification of 2021, the term “diffuse midline glioma, H3 K27-altered” is introduced for this tumor type and is classified as a WHO Grade 4 tumor[^1].