Cushing's syndrome: A study of fifty patients

Abstract

Clinical and laboratory data are reported in fifty patients with Cushing's syndrome. Thirteen patients had adrenal carcinoma, eight a benign adrenal tumor, and in twenty-nine the Cushing's syndrome was associated with non-tumorous adrenal cortical hyperfunction. One or both adrenals were examined in twenty-four patients with non-tumorous adrenal cortical hyperfunction. In thirteen the adrenals were found to be normal in size, in eleven they were enlarged. The presence of an adrenal cortical tumor could be correctly established by perirenal or retrorectal gas insufflation in three-fourths of the tumor group. The incidence of the various clinical signs and symptoms is indicated and the relationship of these manifestations to the underlying adrenal pathology is examined. Some electrolyte abnormality was demonstrable in half of the patients; evidence of disturbance in carbohydrate metabolism was obtained in almost all. The urinary excretion of neutral 17-ketosteroids was increased in half the patients with nontumorous adrenal cortical hyperfunction, onethird of the group with a benign adrenal tumor and two-thirds of those with adrenal carcinoma. All patients had an increase in the urinary excretion of either the formaldehydogenic fractions or the 17-hydroxycorticoids. Seven of the nine patients in whom the plasma level of the 17-hydroxycorticoids was determined demonstrated an increase. The response of the plasma level of the 17-hydroxycorticoids to the intramuscular administration of corticotropin, and also prednisone suppression studies, were of value in establishing the presence or absence of an adrenal tumor before operation. Treatment was determined by the nature of the adrenal pathology. Adrenal tumors were removed surgically, with proper preoperative preparation and postoperative care. In nontumorous adrenal cortical hyperfunction, pituitary radiation, unilateral adrenalectomy, or a combination of both measures, were employed. In approximately 75 per cent of this group a satisfactory remission was obtained. The remaining patients required bilateral adrenalectomy. Treatment was followed by complete disappearance of the clinical and laboratory abnormalities in some cases. The roentgen evidence of osteoporosis, however, remained unaltered, although bone pain subsided and spontaneous fractures no longer occurred. Postmortem studies were made in thirteen patients and the findings are recorded.