Renal Tumors with Pseudohermaphroditism and Glomerular Disease

From 1972 through 1991, 404 patients with renal tumor (Wilms' tumor and clear cell sarcoma) were seen at the Pediatric Oncology Unit of Hacettepe Children's Hospital. The genital abnormalities and renal diseases in these patients were evaluated retrospectively. Eight patients with renal tumor had genital abnormality and/or renal disease of various types and degrees. One of these patients had a clear cell sarcoma while all the others had Wilms' tumor. Two patients had all components of the Drash syndrome. Two patients with Wilms' tumor had genital abnormality and mild proteinuria. Another two patients had only Wilms' tumor and renal disease. Two patients had only renal tumor and genital abnormality of whom one was the patient with clear cell sarcoma.