Pediatric diffuse hemispheric glioma H3 G34-mutant with gains of the BRAF locus: An illustrative case

Rare Tumors. 2023 Apr 7:15:20363613231168704. doi: 10.1177/20363613231168704. eCollection 2023.

Abstract

Diffuse hemispheric glioma, H3 G34-mutant, is a recently recognized distinct high-grade glioma with a dismal prognosis. In addition to the H3 G34 missense mutation, numerous genetic events have been identified in these malignant tumors, including ATRX, TP53, and, rarely, BRAF genes. There are only a few reports to date that have identified BRAF mutations in diffuse hemispheric glioma, H3 G34-mutant. Moreover, to our knowledge, gains of the BRAF locus have yet to be described. Here, we present a case of an 11-year-old male with a diffuse hemispheric glioma, H3 G34-mutant, found to have novel gains of the BRAF locus. Furthermore, we emphasize the current genetic landscape of diffuse hemispheric glioma, H3 G34-mutant, and implications of an aberrant BRAF signaling pathway.

Keywords: Diffuse hemispheric glioma; H3 G34-mutant; H3 K27-mutant; neuro-oncology; pediatric.

Publication types

  • Case Reports