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 Wrensch; Faith G Davis; Bridget J McCarthy; Rose Lai; Beatrice S Melin; Melissa L Bondy; Gliogene Consortium; Michael Kosteljanetz

doi:10.1016/j.ejca.2012.11.009

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, Gliogene Consortium, Michael Kosteljanetz

25 Citations (Scopus)

Abstract

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 language	English
Journal	European journal of cancer (Oxford, England : 1990)
Volume	49
Issue number	6
Pages (from-to)	1335-45
Number of pages	11
ISSN	0959-8049
DOIs	https://doi.org/10.1016/j.ejca.2012.11.009
Publication status	Published - Apr 2013

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Sadetzki, S., Bruchim, R., Oberman, B., Armstrong, G. N., Lau, C. C., Claus, E. B., Barnholtz-Sloan, J. S., Il'yasova, D., Schildkraut, J., Johansen, C., Houlston, R. S., Shete, S., Amos, C. I., Bernstein, J. L., Olson, S. H., Jenkins, R. B., Lachance, D., Vick, N. A., Merrell, R., ... Kosteljanetz, M. (2013). Description of selected characteristics of familial glioma patients – Results from the Gliogene Consortium. European journal of cancer (Oxford, England : 1990), 49(6), 1335-45. https://doi.org/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, Consortium, G & Kosteljanetz, M 2013, 'Description of selected characteristics of familial glioma patients – Results from the Gliogene Consortium', European journal of cancer (Oxford, England : 1990), vol. 49, no. 6, pp. 1335-45. https://doi.org/10.1016/j.ejca.2012.11.009

@article{ea32f6cfe1c04be5be90094b67a98cc7,

title = "Description of selected characteristics of familial glioma patients – Results from the Gliogene Consortium",

abstract = "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.",

author = "Siegal Sadetzki and Revital Bruchim and Bernice Oberman and Armstrong, {Georgina N} and Lau, {Ching C} and Claus, {Elizabeth B} and Barnholtz-Sloan, {Jill S} and Dora Il'yasova and Joellen Schildkraut and Christoffer Johansen and Houlston, {Richard S} and Sanjay Shete and Amos, {Christopher I} and Bernstein, {Jonine L} and Olson, {Sara H} and Jenkins, {Robert B} and Daniel Lachance and Vick, {Nicholas A} and Ryan Merrell and Margaret Wrensch and Davis, {Faith G} and McCarthy, {Bridget J} and Rose Lai and Melin, {Beatrice S} and Bondy, {Melissa L} and Gliogene Consortium and Michael Kosteljanetz",

year = "2013",

month = apr,

doi = "10.1016/j.ejca.2012.11.009",

language = "English",

volume = "49",

pages = "1335--45",

journal = "European Journal of Cancer, Supplement",

issn = "0959-8049",

publisher = "Pergamon",

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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

AU - Consortium, Gliogene

AU - Kosteljanetz, Michael

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.

U2 - 10.1016/j.ejca.2012.11.009

DO - 10.1016/j.ejca.2012.11.009

M3 - Journal article

C2 - 23290425

SN - 0959-8049

VL - 49

SP - 1335

EP - 1345

JO - European Journal of Cancer, Supplement

JF - European Journal of Cancer, Supplement

IS - 6

ER -

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

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