Ectopic Adrenocorticotropic Hormone–Secreting Metastatic Pancreatic Neuroendocrine Tumor Initially Presenting as Gastrinoma: A Case Report

Abstract

Pancreatic neuroendocrine tumors (pNETs) account for 1% to 2% of all pancreatic malignancies; approximately one-third are associated with hormone secretion which can result in a range of symptoms. It is rare for pNETs to transform from secreting one type of peptide hormone to another. We present a case of metastatic pNET that originally presented as a gastrinoma, and later transformed to an ectopic adrenocorticotropic hormone–secreting pNET, treated with bilateral adrenalectomy. We describe the presentation and management of the patient and review the available literature on management of ectopic adrenocorticotropic hormone production in patients with neuroendocrine tumors.