Neuro-oncology practice: Understanding inherited genetic risk of adult glioma - A review

Terri Rice, Daniel H. Lachance, Annette M. Molinaro, Jeanette E. Eckel-Passow, Kyle M. Walsh, Jill Barnholtz-Sloan, Quinn T. Ostrom, Stephen S. Francis, Joseph Wiemels, Robert B. Jenkins, John K. Wiencke, Margaret R. Wrensch

Research output: Contribution to journalArticle

9 Scopus citations

Abstract

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 sixfold 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.

Original languageEnglish (US)
Article numbernpv026
Pages (from-to)1-16
Number of pages16
JournalNeuro-Oncology Practice
Volume3
Issue number1
DOIs
StatePublished - Mar 20 2015

Keywords

  • Epidemiology
  • Genetics
  • Primary adult glioma
  • Risk
  • Variants

ASJC Scopus subject areas

  • Medicine (miscellaneous)

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    Rice, T., Lachance, D. H., Molinaro, A. M., Eckel-Passow, J. E., Walsh, K. M., Barnholtz-Sloan, J., Ostrom, Q. T., Francis, S. S., Wiemels, J., Jenkins, R. B., Wiencke, J. K., & Wrensch, M. R. (2015). Neuro-oncology practice: Understanding inherited genetic risk of adult glioma - A review. Neuro-Oncology Practice, 3(1), 1-16. [npv026]. https://doi.org/10.1093/nop/npv026