The uncovering and characterization of a CCKoma syndrome in enteropancreatic neuroendocrine tumor patients
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The uncovering and characterization of a CCKoma syndrome in enteropancreatic neuroendocrine tumor patients. / Rehfeld, Jens F; Federspiel, Birgitte; Agersnap, Mikkel; Knigge, Ulrich; Bardram, Linda.
In: Scandinavian Journal of Gastroenterology, Vol. 51, No. 10, 2016, p. 1172-1178.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - The uncovering and characterization of a CCKoma syndrome in enteropancreatic neuroendocrine tumor patients
AU - Rehfeld, Jens F
AU - Federspiel, Birgitte
AU - Agersnap, Mikkel
AU - Knigge, Ulrich
AU - Bardram, Linda
PY - 2016
Y1 - 2016
N2 - 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.
AB - 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.
U2 - 10.1080/00365521.2016.1183706
DO - 10.1080/00365521.2016.1183706
M3 - Journal article
C2 - 27191542
VL - 51
SP - 1172
EP - 1178
JO - Scandinavian Journal of Gastroenterology. Supplement
JF - Scandinavian Journal of Gastroenterology. Supplement
SN - 0085-5928
IS - 10
ER -
ID: 177522387