ABSTRACT
Objective To discuss the optimal clinical diagnosis and treatment of ectopic ACTH syndrome with occult tumors. Methods Clinical features, imaging examinations and treatment of 17 patients with ectopic ACTH syndrome were described and compared. Results All patients illustrated the typical clinical features of Cushing’s syndrome. They had hypokalemic alkalosis, elevated serum cortisol and plasma ACTH levels. In the high-dose dexamethasone suppression tests, most patients failed to suppress serum cortisol and 24-hour urinary cortisol. CT and MRI are useful imaging modalities to localize the ACTH-secreting tumor in patients with ectopic ACTH syndrome. The patients with overt ACTH-secreting tumors had surgical curative resection soon after diagnosis. Among patients with occult ACTH-secreting tumors, three underwent subtotal bilateral adrenalectomy, two underwent right adrenalectomy, four received inhibitor of steroidogenesis aminoglutethimide. Their hypercortisolism was controlled. Conclusion Surgical curative resection is the optimal treatment of ectopic ACTH syndrome with overt ACTH-secreting tumor. Bilateral adrenalectomy, right adrenal ectomy or chemotherapy to control hypercortisolism is an available treatment of ectopic ACTH syndrome with occult ACTH-secreting tumors.