The cckOMA syndrome and its relation to the Zollinger–Ellison syndrome: a diagnostic challenge

Abstract

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.

Keywords:

• Cholecystokinin (CCK;)
• CCKoma
• CCKoma syndrome
• diagnostic assays
• gastrin
• gastrinoma
• islet-cell tumor
• neuroendocrine tumors
• Zollinger–Ellison syndrome

Acknowledgement

The highly skilled and patient secretarial assistance of Connie Bundgaard (MA) is most gratefully acknowledged.

Disclosure statement

No potential conflict of interest was reported by the author.

Additional information

Funding

The research behind this review received no external funding.