Department of Oncology, Clinical Sciences, Lund University, Barngatan 2B, 22185, Lund, Sweden.
Fam Cancer. 2012 Mar;11(1):145-55. doi: 10.1007/s10689-011-9493-3.
Whether cancer predisposing familial factors are associated with childhood tumors is unclear. The purpose was to study the incidence of childhood and adult tumors in extended families of children with cancer. Family history of cancer was obtained through questionnaires, and the Swedish Population-, and Cancer Registries for 194 childhood cancer patients aged ≤18 years, diagnosed 1972-2009. Standardized cancer incidence ratios (SIR), and 95% confidence intervals (CI) were estimated and compared with expected rates. Overall, 21 of the 194 patients had any relative with a childhood tumor. When restricted to first- to third degree relatives, increased incidences of childhood (SIR: 2.5; 95% CI: 1.3-4.4) and adult tumors (SIR: 1.5; 95% CI: 1.3-1.7), especially in the prostate (SIR: 2.7; 95% CI: 1.9-3.8) and breast (SIR: 1.7; 95% CI: 1.2-2.4) were observed. Prostate and breast cancers were observed at earlier than average ages. No TP53 mutations or known cancer predisposing syndromes were found in families with multiple childhood tumors. Familial factors may increase the risk for childhood cancer and modify the age of onset of common adult tumors. Studying extended families with multiple childhood tumors may be a valuable approach to understanding the etiology of childhood tumors.
癌症遗传易感性家族因素是否与儿童肿瘤有关尚不清楚。本研究的目的是研究癌症患儿的扩展家族中儿童和成人肿瘤的发病率。通过问卷调查获得癌症家族史，并对 1972 年至 2009 年间诊断为≤18 岁的 194 名儿童癌症患者的瑞典人群和癌症登记处进行了研究。估计并比较了标准化癌症发病率比（SIR）和 95%置信区间（CI）与预期比率。总体而言，194 名患者中有 21 名有亲属患有儿童肿瘤。当仅限于一级至三级亲属时，儿童肿瘤（SIR：2.5；95%CI：1.3-4.4）和成人肿瘤（SIR：1.5；95%CI：1.3-1.7）的发病率增加，尤其是前列腺（SIR：2.7；95%CI：1.9-3.8）和乳房（SIR：1.7；95%CI：1.2-2.4）。前列腺癌和乳腺癌的发病年龄早于平均年龄。在具有多个儿童肿瘤的家庭中未发现 TP53 突变或已知的癌症易感性综合征。家族因素可能会增加儿童癌症的风险，并改变常见成人肿瘤的发病年龄。研究具有多个儿童肿瘤的扩展家族可能是了解儿童肿瘤病因的一种有价值的方法。
