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

Publication ,  Journal Article
Sadetzki, S; Bruchim, R; Oberman, B; Armstrong, GN; Lau, CC; Claus, EB; Barnholtz-Sloan, JS; Il'yasova, D; Schildkraut, J; Johansen, C; Lai, R ...
Published in: Eur J Cancer
April 2013

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.

Duke Scholars

Published In

Eur J Cancer

DOI

EISSN

1879-0852

Publication Date

April 2013

Volume

49

Issue

6

Start / End Page

1335 / 1345

Location

England

Related Subject Headings

  • Young Adult
  • United States
  • Sweden
  • Sex Factors
  • Prevalence
  • Pedigree
  • Oncology & Carcinogenesis
  • Neoplasm Grading
  • Middle Aged
  • Male
 

Citation

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MLA
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Sadetzki, S., Bruchim, R., Oberman, B., Armstrong, G. N., Lau, C. C., Claus, E. B., … Gliogene Consortium, . (2013). Description of selected characteristics of familial glioma patients - results from the Gliogene Consortium. Eur J Cancer, 49(6), 1335–1345. https://doi.org/10.1016/j.ejca.2012.11.009
Sadetzki, Siegal, Revital Bruchim, Bernice Oberman, Georgina N. Armstrong, Ching C. Lau, Elizabeth B. Claus, Jill S. Barnholtz-Sloan, et al. “Description of selected characteristics of familial glioma patients - results from the Gliogene Consortium.Eur J Cancer 49, no. 6 (April 2013): 1335–45. https://doi.org/10.1016/j.ejca.2012.11.009.
Sadetzki S, Bruchim R, Oberman B, Armstrong GN, Lau CC, Claus EB, et al. Description of selected characteristics of familial glioma patients - results from the Gliogene Consortium. Eur J Cancer. 2013 Apr;49(6):1335–45.
Sadetzki, Siegal, et al. “Description of selected characteristics of familial glioma patients - results from the Gliogene Consortium.Eur J Cancer, vol. 49, no. 6, Apr. 2013, pp. 1335–45. Pubmed, doi:10.1016/j.ejca.2012.11.009.
Sadetzki S, 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, Gliogene Consortium. Description of selected characteristics of familial glioma patients - results from the Gliogene Consortium. Eur J Cancer. 2013 Apr;49(6):1335–1345.
Journal cover image

Published In

Eur J Cancer

DOI

EISSN

1879-0852

Publication Date

April 2013

Volume

49

Issue

6

Start / End Page

1335 / 1345

Location

England

Related Subject Headings

  • Young Adult
  • United States
  • Sweden
  • Sex Factors
  • Prevalence
  • Pedigree
  • Oncology & Carcinogenesis
  • Neoplasm Grading
  • Middle Aged
  • Male