Understanding inherited genetic risk of adult glioma - a review.

Journal Article (Journal Article)

During the past six years, researchers have made major progress identifying common inherited genetic variation that increases risk for primary adult glioma. This paper summarizes knowledge about rare familial cancer syndromes that include adult glioma and reviews the available literature on the more recently discovered common inherited variation. Ten independent inherited variants in eight chromosomal regions have been convincingly associated with increased risk for adult glioma. Most of these variants increase relative risk of primary adult glioma by 20% to 40%, but the TP53 variant rs78378222 confers a two-fold relative risk (ie, 200%), and rs557505857 on chromosome 8 confers a six-fold relative risk of IDH-mutated astrocytomas and oligodendroglial tumors (ie, 600%). Even with a six-fold relative risk, the overall risk of developing adult glioma is too low for screening for the high-risk variant on chromosome 8. Future studies will help clarify which inherited adult glioma risk variants are associated with subtypes defined by histology and/or acquired tumor mutations. This review also provides an information sheet for primary adult glioma patients and their families.

Full Text

Duke Authors

Cited Authors

  • Rice, T; Lachance, DH; Molinaro, AM; Eckel-Passow, JE; Walsh, KM; Barnholtz-Sloan, J; Ostrom, QT; Francis, SS; Wiemels, J; Jenkins, RB; Wiencke, JK; Wrensch, MR

Published Date

  • March 2016

Published In

Volume / Issue

  • 3 / 1

Start / End Page

  • 10 - 16

PubMed ID

  • 26941959

Pubmed Central ID

  • PMC4774334

International Standard Serial Number (ISSN)

  • 2054-2577

Digital Object Identifier (DOI)

  • 10.1093/nop/npv026


  • eng

Conference Location

  • England