Diffuse midline glioma H3-K27-altered (WHO grade 4)

Last edit by Alaric Steinmetz on

Synonyms: Diffuse midline glioma, H3 K27-altered

ICD-10: C71.9

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].

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