STEROID SYNTHESIS IN A HUMAN VIRILISING ADRENAL CARCINOMA – SOME UNUSUAL FEATURES
ABSTRACT A patient presenting at the Universitäts-Frauenklinik, Berlin with a long history of increasing hirsutism and virilism provided an opportunity to study an unusual form of adrenal hyperfunction. It was found that she behaved abnormally with respect to the dexamethasone suppression test since her highly elevated 17-oxosteroid output further increased by approximately 400% with 8 mg/day dosage. Her basal 17-hydroxycorticosteroid output was elevated but not grossly so, nor did she exhibit the usual signs of Cushing's syndrome of weight increase and florid complexion. At operation a »tennis ball« size adrenal carcinoma was removed from the left side. Incubation studies were performed on sliced tumour tissue with substrates [1-14C] acetate, [7α-3H] pregnenolone, [4-14C] progesterone and [4-14C] androst-4-ene-3,17-dione. The frozen incubation mixtures were subsequently flown to Cardiff for steroid analysis by reverse radioisotope dilution techniques. The combined urinary and incubation results suggested that, in vivo, the tumour secreted DHA and not DHA sulphate and that there appeared to be a considerable deficiency of DHA-3β-hydroxysteroid dehydrogenase/Δ5−4-isomerase and of DHA-3β-sulphokinase activities although the steroid-11β-hydroxylase, 17α-hydroxylase and 21-hydroxylase activities seemed unimpaired.