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.