title: Genitourinary tumors in the families of children with renal tumors.
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.
authors.affiliation: CRC Paediatric and Familial Cancer Research Group, Christie Hospital Trust, Manchester, England.
authors.forename: A L
authors.initials: AL
authors.lastname: Hartley
authors.forename: J M
authors.initials: JM
authors.lastname: Birch
authors.forename: V
authors.initials: V
authors.lastname: Blair
authors.forename: A M
authors.initials: AM
authors.lastname: Kelsey
authors.forename: M
authors.initials: M
authors.lastname: Harris
authors.forename: P H
authors.initials: PH
authors.lastname: Jones
authors.forename: H R
authors.initials: HR
authors.lastname: Gattamaneni
mesh_terms: D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D016162:Genes, Wilms Tumor; D006801:Humans; D007223:Infant; D007621:Karyotyping; D007680:Kidney Neoplasms / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics; D014565:Urogenital Neoplasms / Q000235:genetics*; D009396:Wilms Tumor / Q000235:genetics