Description of selected characteristics of familial glioma patients-Results from the Gliogene Consortium

Siegal Sadetzki*, Revital Bruchim, Bernice Oberman, Georgina N. Armstrong, Ching C. Lau, Elizabeth B. Claus, Jill S. Barnholtz-Sloan, Dora Il'Yasova, Joellen Schildkraut, Christoffer Johansen, Richard S. Houlston, Sanjay Shete, Christopher I. Amos, Jonine L. Bernstein, Sara H. Olson, Robert B. Jenkins, Daniel Lachance, Nicholas A. Vick, Ryan Merrell, Margaret WrenschFaith G. Davis, Bridget J. McCarthy, Rose Lai, Beatrice S. Melin, Melissa L. Bondy

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

32 Scopus citations


Background: While certain inherited syndromes (e.g. Neurofibromatosis or Li-Fraumeni) are associated with an increased risk of glioma, most familial gliomas are non-syndromic. This study describes the demographic and clinical characteristics of the largest series of non-syndromic glioma families ascertained from 14 centres in the United States (US), Europe and Israel as part of the Gliogene Consortium. Methods: Families with 2 or more verified gliomas were recruited between January 2007 and February 2011. Distributions of demographic characteristics and clinical variables of gliomas in the families were described based on information derived from personal questionnaires. Findings: The study population comprised 841 glioma patients identified in 376 families (9797 individuals). There were more cases of glioma among males, with a male to female ratio of 1.25. In most families (83%), 2 gliomas were reported, with 3 and 4 gliomas in 13% and 3% of the families, respectively. For families with 2 gliomas, 57% were among 1st-degree relatives, and 31.5% among 2nd-degree relatives. Overall, the mean (±standard deviation [SD]) diagnosis age was 49.4 (± 18.7) years. In 48% of families with 2 gliomas, at least one was diagnosed at <40 y, and in 12% both were diagnosed under 40 y of age. Most of these families (76%) had at least one grade IV glioblastoma multiforme (GBM), and in 32% both cases were grade IV gliomas. The most common glioma subtype was GBM (55%), followed by anaplastic astrocytoma (10%) and oligodendroglioma (8%). Individuals with grades I-II were on average 17 y younger than those with grades III-IV. Interpretation: Familial glioma cases are similar to sporadic cases in terms of gender distribution, age, morphology and grade. Most familial gliomas appear to comprise clusters of two cases suggesting low penetrance, and that the risk of developing additional gliomas is probably low. These results should be useful in the counselling and clinical management of individuals with a family history of glioma.

Original languageEnglish
Pages (from-to)1335-1345
Number of pages11
JournalEuropean Journal of Cancer
Issue number6
StatePublished - Apr 2013


FundersFunder number
Tug McGraw Foundation
National Institutes of Health
National Cancer InstituteR01CA119215
American Brain Tumor Association
National Brain Tumor Society


    • Clinical characteristics
    • Familial glioma
    • Genetic counselling
    • Glioma


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