Objective: To investigate and discuss the diagnosis and treatment of ectopic ACTH syndrome.
Methods: Clinical data of 32 cases of ectopic ACTH syndrome, recruited from January 1990 to April 2003 in our hospital, was analyzed.
Results: All of the 32 cases presented with clinical and biochemical evidences of Cushing's syndrome. Ten cases were definitively diagnosed as ectopic ACTH syndrome by finding ectopic tumors; 4 cases were highly suspected as ectopic ACTH by blood sampling from femoral vein and infra-petrosal vein and 18 cases were suspected as ectopic ACTH by imaging examinations. Fifteen cases (47%), without identified source of ectopic hormone, were treated with bilateral or unilateral total adrenalectomy, with 1-year survival rate of 60%. Seven cases (22%), with possible source of ectopic hormone, underwent no intervention, with 1-year survival rate of 0. Ten cases underwent radical resection of tumor, 6 of which were bronchial carcinoids and 4 of which were thymic carcinoids, with 1-year survival rate of 60%.
Conclusion: It is very difficult to localize the tumor of ectopic ACTH syndrome patients. Bilateral adrenalectomy followed by hormonal replacement is effective for most of the patients without identifying source of ectopic hormone.