CUSHING'S SYNDROME DUE TO ADRENOCORTICAL CARCINOMA A COMPHRENSIVE CLINICAL AND BIOCHEMICAL STUDY OF PATIENTS TREATED BY SURGERY AND CHEMOTHERAPY

Abstract

Four post-menopausal women had Cushing''s syndrome due to adrenal cortical carcinomas. Comprehensive analyses of blood and urinary steroids showed that although the steroid profiles differed between patients, the pattern in each patient remained almost constant as the disease progessed or remitted due to therapy. Elevations of serum testosterone and estradiol were commensurate with the extent of virilization and the urinary output of aldosterone was associated with the severity of hypertension. A new finding was that all had substantially increased urinary free deoxycorticosterone. Complete surgical removal of the primary tumors was impossible but when most of the tumor tissue was removed, full clinical and biochemical remissions were obtained for a short time in 2 patients. One patient obtained a clinical and biochemical remission from op''DDD. In another patient the drug caused reduction both in blood pressure and in urinary aldosterone excretion, but there were unpleasant side effects. A 3rd patient could not tolerate op''DDD. Metyrapone therapy produced neither clinical nor biochemical improvement in 3 patients. The mean duration of survival was 17 mo. after the 1st symptoms and 10 mo. from the date of operation. Despite advances in drug therapy, adrenal cortical carcinoma remains a lethal disease. Biochemical screening of multiple steroids offers a means of early diagnosis and disease monitoring. Extensive surgical removal of the tumor offers the best chance of a clinical and biochemical remission.