Ectopic ACTH syndrome caused by hepatic metastases from previously non-functional pancreatic neuroendocrine tumor

Abstract

Background: Pancreatic neuroendocrine tumors (NETs) are rare; representing less than 10% of all primary pancreatic tumors. The minority of these tumors are functional producing a clinical syndrome according to the hormone secreted. ACTH production by pancreatic NETs is rare and even rarer, it occurs from liver metastasis later in the course of the disease. Case Report: We report a case of a 30-year-old Libyan man who presented with Cushing's syndrome due to ectopic ACTH production from liver metastases of a pancreatic NET. The pancreatic NET was diagnosed 7 years previously when it was considered a nonfunctional at the time of diagnosis; the tumor was resected completely then. In the current presentation there was no evidence of the primary tumor but multiple liver metastases were present. Conclusions: This report highlights the fact that initially non-functional pancreatic NETs may present later with a functional hormonal syndrome either from the primary tumor itself or from its metastatic secondary tumors elsewhere.