Endocrine studies in an arrhenoblastoma responsive to dexamethasone, ACTH and human chorionic gonadotropin

Urinary and plasma steroid studies were performed in a 19 year old girl before and after removal of a virilizing arrhenoblastoma. Prior to surgery, base line urinary 17-ketosteroid (17-KS) excretion levels were slightly elevated whereas the plasma testosterone level was markedly elevated and in the normal male range. Individual 17-KS levels were within the normal range, but there was an abnormal androsterone to etiocholanolone ratio. Dexamethasone administration resulted in a fall in plasma testosterone levels and a decrease in urinary 17-KS excretion. The administration of both human chorionic gonadotrophin (HCG) and ACTH was associated with impressive increments in plasma testosterone and/or urinary 17-KS excretion, whereas the administration of HCG alone resulted in increments in only the 11-deoxy 17-KS fractions; ACTH alone raised both the 11-deoxy and 11-oxy 17-KS fractions, with the most notable changes occurring in the former. Within 1 week of surgery, the plasma testosterone level had fallen to normal and urinary 17-KS excretion to subnormal levels. The pre- and postoperative peripheral blood and urinary studies, and the finding of an elevated ovarian vein testosterone level on the side draining the tumor at the time of surgery, demonstrate testosterone secretion by the tumor and, for the first time, an arrhenoblastoma unique in its hormonal responsiveness to dexamethasone, HCG and ACTH.