Breslow N, Olshan A, Beckwith J B, Green D M
Department of Biostatistics, University of Washington, Seattle 98195.
Med Pediatr Oncol. 1993;21(3):172-81. doi: 10.1002/mpo.2950210305.
Wilms tumor affects approximately one child per 10,000 worldwide before the age of 15 years. Incidence rates appear to be slightly elevated for U.S. and African Blacks in comparison to Whites, but are only half as great among Asians. Several case-control studies have suggested that paternal occupational or maternal hormonal exposures during pregnancy may increase the risk of Wilms tumor, but small numbers of subjects and inconsistencies in the patterns of exposures do not permit firm conclusions to be drawn. It is unlikely that such environmental exposures play a major role in the etiology of Wilms tumor. The median age-at-onset of Wilms tumor is 38 months in the U.S. National Wilms Tumor Study series, with cases in girls occurring on average 6 months later than in boys. Patients with bilateral tumors, aniridia, cryptorchism/hypospadias, Beck-with-Wiedemann syndrome, or intralobar nephrogenic rests tend to be diagnosed much younger than average (median 17-27 months). Those with familial disease or multicentric tumors have intermediate age-at-onset distributions, while those with perilobar nephrogenic rests are diagnosed at older ages. The epidemiologic features suggest that somatic mosaicism, rather than a germline mutation, may be responsible for some of the bilateral and multicentric cases.
在全球范围内，15岁前每10000名儿童中约有1名受肾母细胞瘤影响。与白人相比，美国黑人和非洲黑人的发病率似乎略有升高，但在亚洲人中发病率仅为白人的一半。几项病例对照研究表明，孕期父亲的职业暴露或母亲的激素暴露可能会增加肾母细胞瘤的风险，但研究对象数量较少且暴露模式不一致，无法得出确凿结论。此类环境暴露在肾母细胞瘤病因中不太可能起主要作用。在美国国家肾母细胞瘤研究系列中，肾母细胞瘤的中位发病年龄为38个月，女孩发病平均比男孩晚6个月。患有双侧肿瘤、无虹膜、隐睾症/尿道下裂、贝克威思-维德曼综合征或叶内肾源性残留的患者往往比平均年龄诊断得早得多（中位年龄17 - 27个月）。患有家族性疾病或多中心肿瘤的患者发病年龄分布处于中间水平，而患有叶旁肾源性残留的患者诊断年龄较大。流行病学特征表明，体细胞镶嵌现象而非种系突变可能是一些双侧和多中心病例的病因。
