Scand J Gastroenterol
c Department of Surgical Gastroenterology, Rigshospitalet , University of Copenhagen, Copenhagen , Denmark ;
Published: October 2016
Objective: Neuroendocrine tumors in the pancreas and the gastrointestinal tract may secrete hormones which cause specific syndromes. Well-known examples are gastrinomas, glucagonomas, and insulinomas. Cholecystokinin-producing tumors (CCKomas) have been induced experimentally in rats, but a CCKoma syndrome in man has remained unknown until now.
Material And Methods: Using a panel of immunoassays for CCK peptides and proCCK as well as for chromogranin A, we have examined plasma samples from 284 fasting patients with gastroenteropancreatic neuroendocrine tumors. In hyperCCKemic samples, plasma CCK was further characterized by chromatography.
Results: One of the patients displayed gross hyperCCKemia. She was a 58-year old woman with a pancreatic endocrine tumor, liver metastases, 500-1000-fold elevated basal CCK concentration in plasma, diarrhea, severe weight loss, recurrent peptic ulcer and bilestone attacks from a contracted gallbladder. The CCK concentrations in plasma were not affected by resection of the pancreatic tumor, but decreased to normal after hemihepatectomy with removal of the metastases.
Conclusion: A CCKoma syndrome with severe hypersecretion of CCK exists in man. The duodenal ulcer disease and diarrhea with permanently low gastrin in plasma suggest that CCKomas may mimic gastrinoma-like symptoms, because CCK peptides are full agonists of the gastrin/CCK-B receptor.
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http://dx.doi.org/10.1080/00365521.2016.1183706 | DOI Listing |
Scand J Gastroenterol
May 2024
Department of Clinical Biochemistry, University of Copenhagen, Rigshospitalet, Copenhagen, Denmark.
Front Endocrinol (Lausanne)
April 2017
Department of Clinical Biochemistry, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
Cholecystokinin (CCK) was discovered in 1928 in jejunal extracts as a gallbladder contraction factor. It was later shown to be member of a peptide family, which are all ligands for the CCK and CCK receptors. CCK peptides are known to be synthetized in small intestinal endocrine I-cells and cerebral neurons.
View Article and Find Full Text PDFScand J Gastroenterol
October 2016
c Department of Surgical Gastroenterology, Rigshospitalet , University of Copenhagen, Copenhagen , Denmark ;
Objective: Neuroendocrine tumors in the pancreas and the gastrointestinal tract may secrete hormones which cause specific syndromes. Well-known examples are gastrinomas, glucagonomas, and insulinomas. Cholecystokinin-producing tumors (CCKomas) have been induced experimentally in rats, but a CCKoma syndrome in man has remained unknown until now.
View Article and Find Full Text PDFBest Pract Res Clin Endocrinol Metab
January 2016
Yale University School of Medicine, New Haven, CT, USA.
The discovery of neuroendocrine tumours of the gastrointestinal tract and pancreas started in 1870, when Rudolf Heidenhain discovered the neuroendocrine cells, which can lead to the development of these tumours. Siegfried Oberndorfer was the first to introduce the term carcinoid in 1907. The pancreatic islet cells were first described in 1869 by Paul Langerhans.
View Article and Find Full Text PDFArch Surg
September 1994
Department of Surgery, University of California-San Francisco.
Objective: To review our experiences in order to high-light some important lessons learned in the treatment of patients with neuroendocrine gut neoplasms.
Design: Retrospective analysis of case series of 70 patients with neuroendocrine gut neoplasms treated between 1983 and 1993. The clinical features of individual patients illustrate lessons in surgical treatment.
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