Soonchunhyang Med Sci.  2012 Dec;18(2):159-162.

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

Affiliations
  • 1Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea. hkpark@schmc.ac.kr
  • 2Department of Pathology, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea.

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.

Keyword

Adrenocorticotropic hormone; Neuroendocrine tumors; Cushing syndrome

MeSH Terms

Abdomen
Adrenocorticotropic Hormone
Consensus
Cushing Syndrome
Female
Gastrins
Glucagon
Humans
Insulin
Islets of Langerhans
Liver
Neoplasm Metastasis
Neuroendocrine Tumors
Peptides
Prognosis
Spleen
Stomach
Adrenocorticotropic Hormone
Gastrins
Glucagon
Insulin
Peptides
Full Text Links
  • SMS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr