Cancer in siblings of children with cancer in the Nordic countries:: a population-based cohort study
Cancer in siblings of children with cancer in the Nordic countries:: a population-based cohort study
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DOI：
10.1016/s0140-6736(01)05838-x
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发表时间：
2001-09-01
期刊：
影响因子：
168.9
通讯作者：
Olsen, JH
中科院分区：
文献类型：
--
作者：
Winther, JF;Sankila, R;Olsen, JH
在一些罕见的遗传性疾病中，如Li-Fraumeni综合征，癌症患儿的亲属患癌症的风险增加。我们的目的是评估儿童癌症与兄弟姐妹风险之间的关系，并评估隐性条件在癌症病因中的影响。方法：我们在北欧国家进行了一项以人群为基础的队列研究，纳入了25 605名癌症儿童的42 277名兄弟姐妹。从北欧五个癌症登记处的记录中确定患有癌症的儿童，并从全国人口登记处确定他们的兄弟姐妹。通过与癌症登记处的记录联系记录兄弟姐妹的癌症，并与全国发病率进行比较。我们还评估了父母的癌症发病率，以确定家族性癌症综合征。结果：兄弟姐妹中预计有284.2例癌症，而确诊为353例（标准化发病率为1.24,95% CI为1.12-1.38）。兄弟姐妹患病风险比在10岁前最高（2.59,1.89-3.46）。我们排除了56个与癌症相关的遗传综合征家族，将20岁以下兄弟姐妹的比率从1.7降至1.0(0.7-1.3)，将20-29岁兄弟姐妹的比率从1.3降至1.0（0.8-1.3）。我们没有发现家族性癌症的新模式表明遗传易感性，也没有证据表明隐性条件可能导致不能用综合征解释的癌症。在20岁之前发生的兄弟姐妹癌症中，40%可归因于已知的遗传因素，而60%仍无法解释。除了罕见的癌症综合征外，儿科癌症不是兄弟姐妹癌症风险增加的指标。
Background In some rare inherited disorders such as Li-Fraumeni syndrome, relatives of children with cancer are at increased risk of cancer. We aimed to assess relations between childhood cancer and sibling risk, and evaluate the influence of recessive conditions in cancer causation.Methods We did a population-based cohort study in the Nordic countries of 42 277 siblings of 25 605 children with cancer. Children with cancer were identified from records in the five Nordic cancer registries, and their siblings from nationwide population registries. Cancers in siblings were documented through record linkage with cancer registries and compared with national incidence rates. We also assessed cancer incidence in parents to identify familial cancer syndromes.Findings 284.2 cancers were expected in siblings, whereas 353 were diagnosed (standardised incidence ratio 1.24 95% CI 1.12-1.38). Risk ratios for siblings were highest in the first decade of life (2.59, 1.89-3.46). We excluded 56 families with genetic syndromes linked to cancer, which reduced this ratio from 1.7 to 1.0 (0.7-1.3) for siblings younger than 20 years, and from 1.3 to 1.0 (0.8-1.3) for those aged 20-29 years. We found no new patterns of familial cancer that indicated inherited susceptibility, or evidence that recessive conditions might contribute to cancers not explained by syndromes. 40% of cancers in siblings that occurred before age 20 years could be attributed to known genetic factors, whereas 60% remained unexplained.Interpretation Apart from rare cancer syndromes, paediatric cancer is not an indicator of increased cancer risk in siblings.