GENITOURINARY TUMORS IN THE FAMILIES OF CHILDREN WITH RENAL TUMORS

The occurrence of genitourinary tumors in the relatives of a populatio n-based series of 218 children diagnosed with renal tumors was investi gated. Family data on 92% (176 of 192) of Wilms' tumor (WT) patients a nd 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, altho ugh none of these manifested familial WT, It is proposed that a small proportion of families of children with renal tumors has a genetic pre disposition 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.