GONADAL TUMORS IN DISORDERS OF SEXUAL-DIFFERENTIATION

Objective. To study the prevalence and distribution of gonadal tumors in patients with disorders of sexual differentiation. Methods. Retrosp ective review of pathologic materials and clinical data on all patient s diagnosed with mixed gonadal dysgenesis, pure gonadal dysgenesis, an drogen insensitivity, and true hermaphroditism between 1982 and 1990. Results. Twenty-one patients were identified and all underwent bilater al gonadectomy at the time of diagnosis. Nine of 21 patients had a gon adal tumor for a prevalence of 44 percent. Those at greatest risk for tumor were patients with mixed gonadal dysgenesis (6 of 11 patients) a nd pure gonadal dysgenesis (2 of 3 patients). There were four gonadobl astomas, two dysgerminomas, and one each of teratocarcinoma, seminoma, cystadenofibroma, and juvenile granulosa cell tumor. Conclusions. The high prevalence of gonadal tumors in children with mixed and pure gon adal dysgenesis warrants consideration of early, bilateral, prophylact ic gonadectomy once the diagnosis is established with certainty.