ReviewONCOLOGY

Mutations in chromatin machinery and pediatric high-grade glioma

Science Advances  18 Mar 2016:
Vol. 2, no. 3, e1501354
DOI: 10.1126/sciadv.1501354

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Abstract

Pediatric central nervous system tumors are the most common solid tumor of childhood. Of these, approximately one-third are gliomas that exhibit diverse biological behaviors in the unique context of the developing nervous system. Although low-grade gliomas predominate and have favorable outcomes, up to 20% of pediatric gliomas are high-grade. These tumors are a major contributor to cancer-related morbidity and mortality in infants, children, and adolescents, with long-term survival rates of only 10 to 15%. The recent discovery of somatic oncogenic mutations affecting chromatin regulation in pediatric high-grade glioma has markedly improved our understanding of disease pathogenesis, and these findings have stimulated the development of novel therapeutic approaches targeting epigenetic regulators for disease treatment. We review the current perspective on pediatric high-grade glioma genetics and epigenetics, and discuss the emerging and experimental therapeutics targeting the unique molecular abnormalities present in these deadly childhood brain tumors.

Keywords
  • Histone mutation
  • K27M
  • G34V/R
  • pediatric high-grade glioma
  • DIPG
  • methyltransferase
  • demethylase
  • PRC2
  • JMJD3
  • GSKJ4

This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial license, which permits use, distribution, and reproduction in any medium, so long as the resultant use is not for commercial advantage and provided the original work is properly cited.

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