[Intersexuality and malignancy. Case report of an unreported association between a malignant disease in a child with androgen resistance syndrome in comparison with the most frequently expected malignant tumors in children with intersexuality].
Patients with specific disorders in sexual differentiation 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 virilizing 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 leukaemia. This would be to our knowledge the first description of such an unusual association of these two distinct disorders.
Holder
M
M
Olgahospital, Pädiatrisches Zentrum, Stuttgart.
Hecker
W
W
D000728:Androgens / Q000502:physiology*; D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000175:diagnosis / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D006059:Gonadal Dysgenesis / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000235:genetics*; D011944:Receptors, Androgen / Q000235:genetics* / Q000502:physiology; D012074:Remission Induction
D000728:Androgens; D011944:Receptors, Androgen
Intersexualität und Malignität. Kasuistik einer noch nicht beschriebenen Assoziation einer malignen Erkrankung bei einem Kind mit Androgenresistenz-Syndrom im Vergleich zu den am häufigsten zu erwartenden malignen Tumoren bei Kindern mit intersexuellem Genitale.