INTERSEXUALITY AND MALIGNANCY - CASE-REPORT ABOUT AN UNUSUAL ASSOCIATION OF BOTH DISORDERS IN COMPARISON WITH THE MOST FREQUENT MALIGNANT-TUMORS TO BE EXPECTED IN CHILDREN WITH AMBIGUOUS GENITALIA

Patients with specific disorders in sexual differentation have an increased risk for development of malignancies. The most frequent malignant tumors to be expected especially in childhood are gonadoblastoma (carcinoma-in-situ) by children with pure gonadal dysgenesis (Swyer-syndrome) with a calculated risk of approximately 30%. In this disorder the so called streak gonads have an age related, increased risk for development of malignancies. Furthermore, malignant tumors may be expected in children with mixed gonadal dysgenesis (in 10-20%), pure hermaphroditism (in the testes significantly higher than in the ovaries) and with androgen insensitivity syndrome. Risk values for the last are reported in the literature very differently. Rare disorders in this context are virilising tumors of the adrenal cortex, the DRASH-syndrome (nephropathy, most male pseudohermaphroditism, Wilms-tumor) and similar disorders. Practical prophylactic and therapeutic procedures of the separate disorders respectively were discussed. We report about a child with androgen resistance syndrome and acute leucaemia. This would be to our knowledge the first description of such an unusual association of these two distinct disorders.