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

Autor: Sadetzki S; Cancer and Radiation Epidemiology Unit, Gertner Institute, Chaim Sheba Medical Center, Tel Hashomer, Israel. siegals@gertner.health.gov.il, Bruchim R, Oberman B, Armstrong GN, Lau CC, Claus EB, Barnholtz-Sloan JS, Il'yasova D, Schildkraut J, Johansen C, Houlston RS, Shete S, Amos CI, Bernstein JL, Olson SH, Jenkins RB, Lachance D, Vick NA, Merrell R, Wrensch M, Davis FG, McCarthy BJ, Lai R, Melin BS, Bondy ML
Jazyk: angličtina
Zdroj: European journal of cancer (Oxford, England : 1990) [Eur J Cancer] 2013 Apr; Vol. 49 (6), pp. 1335-45. Date of Electronic Publication: 2013 Jan 04.
DOI: 10.1016/j.ejca.2012.11.009
Abstrakt: 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 <40y, and in 12% both were diagnosed under 40y 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 17y 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.
(Copyright © 2012 Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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