Korean J Med.
2011 Feb;80(2):238-242.
An Adrenocorticotropic Hormone-secreting Malignant Pancreatic Neuroendocrine Tumor
- Affiliations
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- 1Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea. oncolee@ewha.ac.kr
- 2Department of Pathology, Ewha Womans University School of Medicine, Seoul, Korea.
- 3Department of Internal Medicine, Inje University College of Medicine, Busan, Korea.
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.
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