Abstract
The classification of intersexuality was determined in 10 patients with an intersex disorder who subsequently suffered a Wilms tumor. Of these 10 patients, 7 (70%) had a disorder of gonadal dysgenesis as follows: 5 had mixed gonadal dysgenesis, 1 had dysgenetic male pseudohermaphroditism and 1 had 46XY pure gonadal dysgenesis. Because the gonads of patients with either of these 3 disorders have a high predilection for the development of testicular neoplasia, the concomitant occurrence of renal tumors in such a patient population suggests that a common defect in the urogenital ridge, the embryonic precursor of the kidneys and gonads, may be the cause of this association.

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