An Adrenocorticotropic Hormone-secreting Malignant Pancreatic Neuroendocrine Tumor.
- Author:
Eun Mi SONG
1
;
Kyoung Eun LEE
;
Jung Youn JO
;
Hea Soo KOO
;
Moon Young CHOI
;
Chu Myong SEONG
;
Soon Nam LEE
Author Information
1. Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea. oncolee@ewha.ac.kr
- Publication Type:Case Report
- Keywords:
ACTH-secreting;
Neuroendocrine tumor;
Pancreas
- MeSH:
Adrenocorticotropic Hormone;
Cushing Syndrome;
Humans;
Islets of Langerhans;
Male;
Middle Aged;
Neoplasm Metastasis;
Neuroendocrine Tumors;
Pancreas;
Prognosis;
Somatostatin
- From:Korean Journal of Medicine
2011;80(2):238-242
- CountryRepublic of Korea
- Language:Korean
-
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.