肾肿瘤患儿家族中的泌尿生殖系统肿瘤

Genitourinary tumors in the families of children with renal tumors.

作者信息

Hartley A L, Birch J M, Blair V, Kelsey A M, Harris M, Jones P H, Gattamaneni H R

机构信息

CRC Paediatric and Familial Cancer Research Group, Christie Hospital Trust, Manchester, England.

出版信息

Cancer Genet Cytogenet. 1994 Jan;72(1):28-32. doi: 10.1016/0165-4608(94)90105-8.

DOI:10.1016/0165-4608(94)90105-8

PMID:8111735

Abstract

The occurrence of genitourinary tumors in the relatives of a population-based series of 218 children diagnosed with renal tumors was investigated. Family data on 92% (176 of 192) of Wilms' tumor (WT) patients and 77% (20 of 26) of other renal tumor patients were obtained. In all, 21 genitourinary tumors in first-degree relatives in 19 families were ascertained, together with 30 such tumors in second-degree relatives. Ten families were diagnosed with multiple genitourinary tumors, although none of these manifested familial WT. It is proposed that a small proportion of families of children with renal tumors has a genetic predisposition to develop genitourinary tumors and that these tumors may represent further manifestations of the pleiotropic effects of the WT1 gene or of other genes involved in WT predisposition.

摘要

对以人群为基础的218例诊断为肾肿瘤的儿童亲属中泌尿生殖系统肿瘤的发生情况进行了调查。获得了92%（192例中的176例）的肾母细胞瘤（WT）患者和77%（26例中的20例）的其他肾肿瘤患者的家族数据。总共确定了19个家庭中一级亲属的21例泌尿生殖系统肿瘤，以及二级亲属中的30例此类肿瘤。10个家庭被诊断患有多发性泌尿生殖系统肿瘤，尽管这些家庭中没有一个表现为家族性WT。有人提出，一小部分肾肿瘤患儿的家庭有发生泌尿生殖系统肿瘤的遗传易感性，并且这些肿瘤可能代表WT1基因或其他与WT易感性相关基因的多效性效应的进一步表现。