The evaluation of the diagnosis and treatment of 32 cases with ectopic ACTH syndrome

Abstract

To investigate and discuss the diagnosis and treatment of ectopic ACTH syndrome. Clinical data of 32 cases of ectopic ACTH syndrome, recruited from January 1990 to April 2003 in our hospital, was analyzed. 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%. 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.