An Uncommon Case of Ectopic Adrenocorticotropic Hormone Syndrome from a Pancreatic Neuroendocrine Tumor

Ectopic adrenocorticotropic hormone (ACTH) syndrome is a rare form of Cushing disease (CD) with over-secretion of ACTH from nonpituitary tumor outside the adrenal or pituitary glands. Its diagnosis relies on both biochemical tests (high-dose dexamethasone suppression test, ACTH level, corticotropin-releasing hormone test) to confirm ACTH-dependent CD and image studies (CT or MRI of chest, abdomen, and/or pelvis) for source localization. We present a rare case of ectopic ACTH syndrome from a pancreatic neuroendocrine tumor (NET).