ADRENAL CORTICAL TUMOR ASSOCIATED WITH CUSHING'S SYNDROME: REPORT OF A CASE WITH METABOLIC STUDIES AND REMARKS ON THE PATHOGENESIS OF CUSHING'S. SYNDROME

Abstract

A case of adrenal cortical tumor has been reported. The patient presented the clinical features of Cushing''s syndrome, including hypertension and hypokalemic, hypochloremic alkalosis. Urinary excretion of both 17-ketosteroids and estrogenic substances was increased to abnormally high levels. The beta fraction of the urinary 17-ketosteroids was increased. Remission of symptoms followed the removal of the tumor. Recurrence of the tumor was accompanied by the reappearance of the clinical picture. Removal of the recurrent tumor was followed by a temporary and incomplete remission of symptoms. Recurrence of the tumor and the appearance of metastatic lesions in the lungs were associated with the symptoms that were present prior to the removal of the tumor. Roentgen therapy proved to be ineffective. At necropsy the following significant observations were made: the anterior pituitary contained 2 small chromophobe tumors; Crooke''s changes were present basophilic cells; the hypothalamus appeared normal; the tumor had recurred locally and had metasta-sized to the lungs; the contralateral adrenal cortex was atrophic; although the patient had osteoporosis, the parathyroids appeared normal. The immediate cause of death was a bleeding duodenal ulcer. The urine contained dehydroisoandrosterone; etiocholan- 3(alpha)-ol-17-one; androsterone; androstane-3(alpha), ll(beta) -diol-17-one; pregnane-3(alpha), 20(alpha)-diol; delta 5-andro-stene-3(beta), 17(alpha)-diol; delta 5-androstene-3(beta), 16 (beta), 17(beta)-triol; estrone and a substance with the empirical formula C19H32O2. Metabolic studies were conducted before the first operation and shortly thereafter. Under the conditions employed, the over-all N balance before operation was negative. Creatine was present in the urine. K was lost from the body and Na was retained when the K content of the diet was kept at a low level. Admn. of KC1 tended to correct the alkalosis, the negative balances for K and N and the positive balance for Na. After removal of the tumor, N, Na, K and chloride all were retained in large amts., and creatinuria practically disappeared. Excretion of urine decreased in amt., and its pH decreased post-operatively. The metabolic data suggested that the functioning adrenal tumor caused loss of muscle and deposition of fat and that its removal was followed by loss of fat and deposition of muscle.