H3-G34-mutant diffuse hemispheric glioma

Synonyms
Diffuse hemispheric glioma, H3 G34-mutant
ICD-10
C71.1

H3-G34-mutant diffuse hemispheric gliomas are characterized by missense mutations in codon 34 of the histone H3 gene H3–3A and are classified as grade 4 tumors according to the 2021 WHO Classification[^1].

WHO Classification

In the 2016 WHO Classification, these gliomas were still classified among glioblastomas, IDH-wildtype. However, these gliomas exhibit distinct molecular and clinical features, including a characteristic DNA methylation pattern, as well as a preferential presentation in the cerebral hemispheres[^2], and were therefore classified as a distinct entity in the 2021 WHO Classification.

Figure

WHO 2021 classification of gliomas
Classification of gliomas according to the WHO classification published in 2021. Figure adapted from Weller et al. 2021.

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