A Case of ACTH-producing Pancreatic Neuroendocrine Tumor Presenting with Cushing Syndrome

ABSTRACT

Pancreatic neuroendocrine tumors are rare neoplasm arising from pancreatic islet cells. Occasionally they are functioning tumors secreting a variety of hormones such as insulin, gastrin, glucagon, and vasoactive intestinal peptides. Adrenocorticotropic hormone (ACTH) secreting pancreatic neuroendocrine tumors (ACTHomas) are very rare and there have been about 110 case reports worldwide. Due to excessive ectopic ACTH production and resulting hypercortisolemia, patients with ACTHoma usually present with Cushing syndrome. ACTHomas have a poor prognosis with severe and rapidly progressive clinical courses. They often present with unresectable liver metastases and there remains little consensus on its standard treatment. We report a 55-year-old female with ACTH-producing pancreatic neuroendocrine tumor, who was treated with surgical resection of pancreatic tail, spleen, and a portion of stomach. Sixteen months later, abdomen computed tomography scan showed multiple liver metastases, which were treated with transarterial chemoembolization.