A scoping review of diffuse hemispheric glioma, H3 G34-mutant: Epigenetic and molecular profiles, clinicopathology, and treatment avenues.

Background: Survival of pediatric and young adults with malignant glioma remains poor despite progress in treatment. This is especially true for diffuse hemispheric glioma (DHG), H3 G34-mutant, which is often present in adolescent and young adult patients. This scoping review consolidates existing knowledge of DHG H3 G34-mutant and identifies future targets and therapeutic options. By streamlining this information, we aim to elucidate knowledge gaps in the field to better inform the community and motivate future research efforts.

Methods: In October 2024, MEDLINE, Embase, Cochrane Library, and Web of Science Core Collection were searched. Two reviewers screened all articles by title and abstract review and 3 independent reviewers extracted all studies meeting inclusion criteria relevant to H3G34R/V tumors (preclinical and clinical studies).

Results: Of the 2203 articles screened, 220 were deemed eligible (79 literature reviews, 7 systematic reviews, 63 preclinical studies, and 71 clinically oriented studies). We found that the United States and Acta Neuropathologica were the top country and journal contributors, respectively.

Conclusion: For this disease, it is critical to the field to conduct further research related to complexities of the tumor microenvironment, translation of preclinical studies to therapeutic early phase trials, and determining the role of targeted central nervous system drug delivery, so as to improve disease prognosis and survival.