title: The uncovering and characterization of a CCKoma syndrome in enteropancreatic neuroendocrine tumor patients.
abstract: 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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: a Department of Clinical Biochemistry, Rigshospitalet , University of Copenhagen , Copenhagen , Denmark ;
authors.forename: Jens F
authors.initials: JF
authors.lastname: Rehfeld
authors.affiliation: b Department of Pathology, Rigshospitalet , University of Copenhagen , Copenhagen , Denmark ;
authors.forename: Birgitte
authors.initials: B
authors.lastname: Federspiel
authors.affiliation: a Department of Clinical Biochemistry, Rigshospitalet , University of Copenhagen , Copenhagen , Denmark ;
authors.forename: Mikkel
authors.initials: M
authors.lastname: Agersnap
authors.affiliation: c Department of Surgical Gastroenterology, Rigshospitalet , University of Copenhagen , Copenhagen , Denmark ;|d Department of Medical Endocrinology, Rigshospitalet , University of Copenhagen , Copenhagen , Denmark.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Knigge
authors.affiliation: c Department of Surgical Gastroenterology, Rigshospitalet , University of Copenhagen , Copenhagen , Denmark ;
authors.forename: Linda
authors.initials: L
authors.lastname: Bardram
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D002648:Child; D002766:Cholecystokinin / Q000097:blood*; D003718:Denmark; D005260:Female; D015408:Gastrinoma; D005755:Gastrins / Q000097:blood*; D006801:Humans; D007414:Intestinal Neoplasms / Q000097:blood* / Q000473:pathology; D008113:Liver Neoplasms / Q000556:secondary*; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000097:blood* / Q000473:pathology; D010179:Pancreas / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000097:blood* / Q000473:pathology; D051381:Rats; D013274:Stomach Neoplasms / Q000097:blood* / Q000473:pathology; D055815:Young Adult
chemical_list: D005755:Gastrins; D002766:Cholecystokinin
keywords: CCKoma; cholecystokinin; gastrin/CCK-B receptor; gastrinomas; neuroendocrine tumor; pancreatic islet-cell tumors