Beheshti M, Mancer J F, Hardy B E, Churchill B M, Bailey J D
Urology. 1984 Aug;24(2):130-3. doi: 10.1016/0090-4295(84)90413-8.
The records of 170 patients with unilateral and 18 patients with bilateral Wilms tumor and 6 patients with congenital mesoblastic nephroma were reviewed for abnormalities of the external genitalia. There were 4 patients with cryptorchism, 1 with hypospadias, 1 with mixed gonadal dysgenesis, and 3 with male pseudohermaphroditism. Of the group, these 9 patients had earlier symptoms; 6 of them were under two years old. Five patients (27.7%) with bilateral Wilms tumors had external genital anomalies in contrast to only 4 (2.3%) of those with unilateral tumors. One of the 9 children with genital anomalies had unilateral congenital mesoblastic nephroma; the others had Wilms tumor. In all cases the histologic subtypes were unusual. These observations substantiate the previously reported association between external genital abnormalities and Wilms tumor and also represent a somewhat higher than expected incidence. The children with this association are remarkable for the high frequency of a particular histologic type of tumor and for the high incidence of bilaterality. The high incidence of Wilms tumors in the male pseudohermaphrodite population presented suggests that such patients should be monitored for development of renal tumors.
回顾了170例单侧肾母细胞瘤患者、18例双侧肾母细胞瘤患者以及6例先天性中胚叶肾瘤患者的外生殖器异常记录。其中有4例隐睾症患者、1例尿道下裂患者、1例混合性性腺发育不全患者以及3例男性假两性畸形患者。在这组患者中，这9例有较早出现的症状；其中6例年龄在两岁以下。双侧肾母细胞瘤患者中有5例（27.7%）存在外生殖器异常，相比之下，单侧肾母细胞瘤患者中仅有4例（2.3%）有此异常。9例有生殖器异常的儿童中，1例患有单侧先天性中胚叶肾瘤；其他患有肾母细胞瘤。所有病例的组织学亚型均不常见。这些观察结果证实了先前报道的外生殖器异常与肾母细胞瘤之间的关联，且其发生率也略高于预期。有这种关联的儿童在特定组织学类型肿瘤的高频率以及双侧性的高发生率方面较为显著。所呈现的男性假两性畸形人群中肾母细胞瘤的高发生率表明，此类患者应监测是否发生肾肿瘤。
