학술논문
Description of selected characteristics of familial glioma patients – Results from the Gliogene Consortium
Document Type
article
Author
Sadetzki, Siegal; Bruchim, Revital; Oberman, Bernice; Armstrong, Georgina N; Lau, Ching C; Claus, Elizabeth B; Barnholtz-Sloan, Jill S; Il’yasova, Dora; Schildkraut, Joellen; Johansen, Christoffer; Houlston, Richard S; Shete, Sanjay; Amos, Christopher I; Bernstein, Jonine L; Olson, Sara H; Jenkins, Robert B; Lachance, Daniel; Vick, Nicholas A; Merrell, Ryan; Wrensch, Margaret; Davis, Faith G; McCarthy, Bridget J; Lai, Rose; Melin, Beatrice S; Bondy, Melissa L; Consortium, Gliogene
Source
European Journal of Cancer. 49(6)
Subject
Language
Abstract
BackgroundWhile 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.MethodsFamilies 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.FindingsThe 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