EPIDEMIOLOGY OF WILMS-TUMOR
EPIDEMIOLOGY OF WILMS-TUMOR
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DOI：
10.1002/mpo.2950210305
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发表时间：
1993-01-01
期刊：
影响因子：
--
通讯作者：
GREEN, DM
中科院分区：
文献类型：
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作者：
BRESLOW, N;OLSHAN, A;GREEN, DM
全世界大约每10,000名儿童中就有一名在15岁之前患上肾母细胞瘤。与白人相比，美国和非洲黑人的发病率似乎略高，但亚洲人的发病率只有白人的一半。一些病例对照研究表明，怀孕期间父亲的职业或母亲的激素暴露可能会增加患肾母细胞瘤的风险，但受试者数量较少，暴露模式不一致，无法得出明确的结论。这种环境暴露不太可能在肾母细胞瘤的病因中起主要作用。在美国国家肾母细胞瘤研究系列中，肾母细胞瘤的发病年龄中位数为38个月，女孩的病例平均比男孩晚6个月。双侧肿瘤、无虹膜、隐睾症/尿道下裂、Beck-with-Wiedemann综合征或叶内肾源性静息的患者往往比平均年龄(中位数17-27个月)年轻得多。有家族性疾病或多中心肿瘤的患者发病年龄分布居中，而有叶周致肾小球病变的患者诊断年龄较大。流行病学特征表明，体细胞嵌合体，而不是胚系突变，可能是导致一些双侧和多中心病例的原因。
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 nephorogenic rests are diagnosed at older ages. The epidemiologic features suggest that somatic mosaicism, rather than a germ-line mutation, may be responsible for some of the bilateral and multicentric cases.