Molecular background of oligodendroglioma: 1p/19q, IDH, TERT, CIC and FUBP1

CNS Oncol. 2015;4(5):287-94. doi: 10.2217/cns.15.32. Epub 2015 Nov 6.

Abstract

Oligodendroglioma is the quintessential molecularly-defined brain tumor. The characteristic whole-arm loss of the long arm of chromosome 1 and the short arm of chromosome 19 (1p/19q-codeletion) within the genome of these tumors facilitated the reproducible molecular identification of this subcategory of gliomas. More recently, recurrent molecular genetic alterations have been identified to occur concurrently with 1p/19q-codeletion, and definitively identify these tumors, including mutations in IDH1/2, CIC, FUBP1, and the TERT promoter, as well as the absence of ATRX and TP53 alterations. These findings provide a foundation for the consistent diagnosis of this tumor type, upon which a generation of clinical investigators have assembled a strong evidence base for the effective treatment of this disease with radiation and chemotherapy.

Keywords: 1p/19q loss; CIC mutation; FUBP1 mutation; IDH mutation; TERT mutation; oligodendroglioma.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Brain Neoplasms / genetics*
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 1 / genetics
  • Chromosomes, Human, Pair 19 / genetics
  • DNA Helicases / genetics
  • DNA-Binding Proteins / genetics
  • Disease Management
  • Epigenomics
  • Humans
  • Isocitrate Dehydrogenase / genetics
  • Mutation / genetics*
  • Oligodendroglioma / genetics*
  • Oligodendroglioma / therapy
  • RNA-Binding Proteins
  • Repressor Proteins / genetics
  • Telomerase / genetics

Substances

  • CIC protein, human
  • DNA-Binding Proteins
  • FUBP1 protein, human
  • RNA-Binding Proteins
  • Repressor Proteins
  • Isocitrate Dehydrogenase
  • TERT protein, human
  • Telomerase
  • DNA Helicases