CLEVELAND CLINIC EXPERIENCE WITH ADRENAL CUSHINGS-SYNDROME

Purpose: Cushing's syndrome due to adrenal adenoma or adrenocortical c arcinoma is rare. To understand better the clinical and biochemical pr esentation of this disorder, as well as therapy efficacy and patient s urvival, we conducted a retrospective review. Materials and Methods: B etween August 1971 and April 1994, 40 patients presented to our instit ution with adrenal Cushing's syndrome (27 adenomas and 13 carcinomas). These groups were analyzed with respect to clinical signs and symptom s preoperatively and postoperatively, biochemical analysis, length of postoperative steroid replacement therapy, disease recurrence and pati ent survival. Followup was obtained by chart review and telephone inte rviews and averaged 59.6 +/- 66.4 and 47.6 +/- 56.2 months for adenoma and carcinoma patients, respectively. Results: Women predominated in both groups (26 of 27 adenomas, 11 of 13 carcinomas), and tumors affec ted the left adrenal gland more frequently (19 of 27 adenomas, 9 of 13 carcinomas). Adenoma patients were younger than carcinoma patients (3 9.6 +/- 14.4 versus 51.5 +/- 16.6 years, p = 0.026) and presented with smaller tumors (3.3 +/- 1.0 versus 8.6 +/- 4.5 cm., p = 0.001). There was a trend toward increased incidence of glucose intolerance among c arcinoma patients but no significant differences in clinical signs or symptoms between adenoma and carcinoma patients could be made. Similar ly, while there was no significant difference in biochemical evaluatio n of adenoma versus carcinoma patients, 24-hour urinary free cortisol and serum lactate dehydrogenase levels tended to be higher among carci noma patients. In addition 17-ketosteroid and dehydroepiandrosterone s ulfate levels were more elevated in carcinoma than in adenoma patients , and several adenoma patients actually had subnormal levels. Among ad enoma patients mean length of steroid replacement therapy was 16.8 +/- 9.1 months. However, 7 adenoma patients (25.9%) required greater than 24 months of exogenous steroids, and only 1 of these patients was sub sequently weaned off steroid replacement. There were no recurrences am ong adenoma patients, although there was 1 perioperative death due to hypoglycemia. Ten (76.9%) carcinoma patients had recurrences at a mean followup of 33 months. The 3 and 5-year survival rates were 41.5 and 31.2%, respectively. Conclusions: While presenting signs and symptoms and hormonal analysis may suggest benign or malignant disease, only tu mor size and patient age are reliable preoperative indicators of adren al adenoma versus adrenocortical carcinoma among patients with adrenal Cushing's syndrome. Surgery is curative for adenoma patients, but lif elong steroid replacement may be required. Survival remains poor among carcinoma patients.