Rahman et al. (1996) described a large Canadian family with 7 confirmed cases of Wilms tumor in 3 generations. No congenital abnormalities or other cancers had been observed in the family. The average age of presentation was 5 ... Rahman et al. (1996) described a large Canadian family with 7 confirmed cases of Wilms tumor in 3 generations. No congenital abnormalities or other cancers had been observed in the family. The average age of presentation was 5 years (age range of 2 to 12 years), which is older than the average age of diagnosis of sporadic WT (3 to 4 years). Typical triphasic histology with stromal, blastemal, and epithelial elements was found in 5 tumors, while the sixth tumor was predominantly myogenic. Five tumors were reported to be unilateral. The myogenic WT was reported to be bilateral at preoperative CT scan. However, at surgery one kidney appeared normal and no biopsy was performed. The predominance of unilateral WTs in this pedigree was consistent with reports from other WT families. Rahman et al. (1998) analyzed the phenotype of 16 WT patients from families linked to the 17q locus. The patients presented at a significantly older age and at a significantly later stage than both patients with sporadic WT and 6 cases from 2 families unlinked to either 17q or 11p13 (WT1).