Rare Tumors (Mar 2023)

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

  • Christine Marlow,
  • Joshua A Cuoco,
  • Austin R Hoggarth,
  • Michael S Stump,
  • Lisa S Apfel,
  • Cara M Rogers

DOI
https://doi.org/10.1177/20363613231168704
Journal volume & issue
Vol. 15

Abstract

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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.