Studies in Man on Gonadotropin-Responsive Feminizing Adrenal Cortical Neoplasia

Abstract

Investigations concerning hormonal responsiveness and steroid hormone biosynthesis are reported in a patient with a feminizing adrenal cortical tumor. Chromatographic separation and quantitation of individual urinary C₁₈, C₁₉ and C₂₁ steroids, and serial measurements of urinary total 17-ketosteroids (17-KS), 17-hydroxycorticosteroids (17-OH) and estrogens were performed before and after the infusion of corticotropin (ACTH), human chorionic gonadotropin (HCG), containing primarily luteinizing hormonelike activity, and pregnant mare's serum gonadotropin (PMS), containing primarily follicle-stimulating hormonelike activity, before and after removal of the tumor. Elevated control levels of urinary total estrogen (predominantly estriol) and 17-KS (predominantly dehydroepiandrosterone) were observed. Total estrogens doubled following HCG, and increased 5-fold after PMS administration; both changes were due to an increase in the estrone fraction. Total 17-KS appeared to increase after PMS, and were unchanged following HCG infusion. There was no appreciable change in urinary C₁₈ and C₁₉ steroids following the administration of ACTH. These data suggest that the steroid biosynthetic pathways in this tumor closely resemble those usually found in normal ovarian tissue; and they are the first emonstration in man of a gonadotropin-responsive adrenal tumor.