Objective: Among patients with enteropancreatic neuroendocrine tumor syndromes only one case with a cholecystokinin (CCK) secreting tumor has been reported. She had significant hyperCCKemia leading to a specific syndrome of severe diarrheas, weight loss, repeated duodenal ulcers and a permanently contracted gallbladder with gallstones. There are, however, reasons to believe that further CCKomas exist, for instance among Zollinger-Ellison patients with normal plasma gastrin concentrations. The present review is a call to gastroenterologists for awareness of such CCKoma patients.
Method: After a short case report, the normal endocrine and oncological biology of CCK is described. Subsequently, the CCKoma symptoms are discussed with particular reference to the partly overlapping symptoms of the Zollinger-Ellison syndrome. In this context, the diagnostic use of truly specific CCK and gastrin assays are emphasized. The discussion also entails the problem of access to accurate CCK measurements.
Conclusion: Obviously, the clinical awareness about the CCKoma syndrome is limited. Moreover, it is also likely that the knowledge about the necessary specificity demands of diagnostic gastrin and CCK assays have obscured proper diagnosis of the CCKoma syndromes in man.
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http://dx.doi.org/10.1080/00365521.2024.2308532 | 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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