Biomedicines (Jul 2023)

H3G34-Mutant Gliomas—A Review of Molecular Pathogenesis and Therapeutic Options

  • Anthony V. Nguyen,
  • Jose M. Soto,
  • Sarah-Marie Gonzalez,
  • Jennifer Murillo,
  • Eric R. Trumble,
  • Frank Y. Shan,
  • Jason H. Huang

DOI
https://doi.org/10.3390/biomedicines11072002
Journal volume & issue
Vol. 11, no. 7
p. 2002

Abstract

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The 2021 World Health Organization Classification of Tumors of the Central Nervous System reflected advances in understanding of the roles of oncohistones in gliomagenesis with the introduction of the H3.3-G34R/V mutant glioma to the already recognized H3-K27M altered glioma, which represent the diagnoses of pediatric-type diffuse hemispheric glioma and diffuse midline glioma, respectively. Despite advances in research regarding these disease entities, the prognosis remains poor. While many studies and clinical trials focus on H3-K27M-altered-glioma patients, those with H3.3-G34R/V mutant gliomas represent a particularly understudied population. Thus, we sought to review the current knowledge regarding the molecular mechanisms underpinning the gliomagenesis of H3.3-G34R/V mutant gliomas and the diagnosis, treatment, long-term outcomes, and possible future therapeutics.

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