A Case of Cushing Syndrome Secondary to Ectopic Adrenocorticotropic Hormone Producing Carcinoid of the Duodenum

Abstract

Cushing syndrome caused by adrenocorticotropic hormone (ACTH) production from solid tumors can result in life-threatening hypercortisolemia. Ectopic ACTH production is most commonly associated with bronchial carcinoids and squamous cell carcinoma of the lung. We report a case of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum. The patient presented to an outside hospital in hypertensive crisis and diabetic ketoacidosis. After stabilization, diagnostic studies including a serum cortisol level, and computed tomography (CT) scans of the head, chest, abdomen, and pelvis revealed hypercortisolemia and a large mass in the head of the pancreas. Pancreaticoduodenectomy was performed. Pathologic investigation revealed a 1-cm carcinoid of the duodenum with two large metastatic lymph nodes near the head of the pancreas. This is the first reported case in the English literature of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum.