INTERSEXUALITY AND MALIGNANCY - CASE-REPO RT 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 incr eased risk for development of malignancies. The most frequent malignan t tumors to be expected especially in childhood are gonadoblastoma (ca rcinoma-in-situ) by children with pure gonadal dysgenesis (Swyer-syndr ome) with a calculated risk of approximately 30%. In this disorder the so called streak gonads have an age related, increased risk for devel opment of malignancies. Furthermore, malignant tumors may be expected in children with mixed gonadal dysgenesis (in 10-20%), pure hermaphrod itism (in the testes significantly higher than in the ovaries) and wit h androgen insensitivity syndrome. Risk values for the last are report ed in the literature very differently. Rare disorders in this context are virilising tumors of the adrenal cortex, the DRASH-syndrome (nephr opathy, most male pseudohermaphroditism, Wilms-tumor) and similar diso rders. Practical prophylactic and therapeutic procedures of the separa te disorders respectively were discussed. We report about a child with androgen resistance syndrome and acute leucaemia. This would be to ou r knowledge the first description of such an unusual association of th ese two distinct disorders.