An Adrenocorticotropic Hormone-secreting Malignant Pancreatic Neuroendocrine Tumor / 대한내과학회지

ABSTRACT

Pancreatic endocrine tumors are low-to-intermediate-grade neoplasms arising from the pancreatic islets that produce various hormones. These tumors account for a minority of pancreatic tumors and are generally considered functional if they are associated with a hormonal syndrome. Adrenocorticotropic hormone-secreting tumors (ACTHomas) are very rare and require special clinical consideration. Patients with ACTH-secreting tumors usually present with Cushing's syndrome, due to ectopic ACTH production. ACTHomas have a poor prognosis because of early metastasis and difficulty controlling corticosteroid production. We report a 62-year-old male with an ACTH-secreting metastatic pancreatic neuroendocrine tumor, who did not respond to somatostatin analogs and died.