TY - JOUR
T1 - Description of selected characteristics of familial glioma patients-Results from the Gliogene Consortium
AU - Sadetzki, Siegal
AU - Bruchim, Revital
AU - Oberman, Bernice
AU - Armstrong, Georgina N.
AU - Lau, Ching C.
AU - Claus, Elizabeth B.
AU - Barnholtz-Sloan, Jill S.
AU - Il'Yasova, Dora
AU - Schildkraut, Joellen
AU - Johansen, Christoffer
AU - Houlston, Richard S.
AU - Shete, Sanjay
AU - Amos, Christopher I.
AU - Bernstein, Jonine L.
AU - Olson, Sara H.
AU - Jenkins, Robert B.
AU - Lachance, Daniel
AU - Vick, Nicholas A.
AU - Merrell, Ryan
AU - Wrensch, Margaret
AU - Davis, Faith G.
AU - McCarthy, Bridget J.
AU - Lai, Rose
AU - Melin, Beatrice S.
AU - Bondy, Melissa L.
N1 - Funding Information:
This work was supported by Grants from the National Institutes of Health , Bethesda, Maryland ( R01 CA119215 01-05 ). Additional support was provided by the American Brain Tumor Association , The National Brain Tumor Society , and the Tug McGraw Foundation . For more information about the Gliogene Consortium, refer to the following Web site: http://www.gliogene.org .
PY - 2013/4
Y1 - 2013/4
N2 - 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.
AB - 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.
KW - Clinical characteristics
KW - Familial glioma
KW - Genetic counselling
KW - Glioma
UR - http://www.scopus.com/inward/record.url?scp=84875733575&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2012.11.009
DO - 10.1016/j.ejca.2012.11.009
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C2 - 23290425
AN - SCOPUS:84875733575
SN - 0959-8049
VL - 49
SP - 1335
EP - 1345
JO - European Journal of Cancer
JF - European Journal of Cancer
IS - 6
ER -
