Familiality in brain tumors

Familiality in brain tumors
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DOI:
10.1212/01.wnl.0000326597.60605.27
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发表时间:
2008-09-23
期刊:
影响因子:
9.9
通讯作者:
Cannon-Albright, Lisa A.
Cannon-Albright, Lisa A.
中科院分区:
医学1区
文献类型:
--
作者:
Blumenthal, Deborah T.;Cannon-Albright, Lisa A.

文献摘要

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背景资料:脑肿瘤的家族性是不明确的substituted.Methods:我们使用的犹他州人口数据库(UMB),家谱代表的犹他州的先驱和他们的后代,记录连接到全州范围内的癌症记录,描述原发性脑癌的家族性。我们研究了原发性脑肿瘤的家族聚集性,包括由组织学类型和诊断时年龄定义的亚组。该研究包括1,401例定义为星形细胞瘤或胶质母细胞瘤的原发性脑肿瘤病例,所有病例均具有至少三代家谱数据。我们使用家系指数方法检验了脑肿瘤病例过度相关性的假设。我们估计脑肿瘤的相对风险在亲属使用率的脑肿瘤internal.Results估计:观察到的星形细胞瘤和胶质母细胞瘤作为一个组(n = 1,401),星形细胞瘤单独考虑(n = 744),但不是胶质母细胞瘤单独考虑(n = 658)。星形细胞瘤的一级和二级亲属患星形细胞瘤的风险显著增加,这与单独考虑星形细胞瘤的亲属有关。星形细胞瘤和胶质母细胞瘤病例一起考虑,胶质母细胞瘤病例单独考虑,一级亲属的风险显著增加,但二级亲属的风险没有显著增加。有证据表明,这种家庭方面不仅包括共享的环境,而且还包括遗传的成分。扩展的高风险脑肿瘤家系中确定的CNOB可能提供了机会,以确定易感基因负责家族性脑肿瘤。
Background: Familiality in brain tumors is not definitively substantiated.Methods: We used the Utah Population Data Base (UPDB), a genealogy representing the Utah pioneers and their descendants, record-linked to statewide cancer records, to describe the familial nature of primary brain cancer. We examined the familial clustering of primary brain tumors, including subgroups defined by histologic type and age at diagnosis. The UPDB includes 1,401 primary brain tumor cases defined as astrocytoma or glioblastoma, all with at least three generations of genealogy data. We tested the hypothesis of excess relatedness of brain tumor cases using the Genealogical Index of Familiality method. We estimated relative risks for brain tumors in relatives using rates of brain tumors estimated internally.Results: Significant excess relatedness was observed for astrocytomas and glioblastomas considered as a group (n = 1,401), for astrocytomas considered separately (n = 744), but not for glioblastomas considered separately (n = 658). Significantly increased risks to first- and second-degree relatives for astrocytomas were identified for relatives of astrocytomas considered separately. Significantly increased risks to first- degree relatives, but not second degree, were observed for astrocytoma and glioblastoma cases considered together, and for glioblastoma cases considered separately.Conclusions: This study provides strong evidence for a familial contribution to primary brain cancer risk. There is evidence that this familial aspect includes not only shared environment, but also a heritable component. Extended high-risk brain tumor pedigrees identified in the UPDB may provide the opportunity to identify predisposition genes responsible for familial brain tumors.