cholecystokinin and Gastrinoma

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.. 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.. 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.. 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.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Animals; Child; Cholecystokinin; Denmark; Female; Gastrinoma; Gastrins; Humans; Intestinal Neoplasms; Liver Neoplasms; Male; Middle Aged; Neuroendocrine Tumors; Pancreas; Pancreatic Neoplasms; Rats; Stomach Neoplasms; Young Adult

To review our experiences in order to high-light some important lessons learned in the treatment of patients with neuroendocrine gut neoplasms.. 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.. University hospitals with tertiary care referral practice.. The main intervention was abdominal exploration in 43 patients, with resection of the primary tumor in 39 and of hepatic metastases in four.. To describe the tumors seen and to identify major lessons learned.. Of 70 patients with neuroendocrine tumors treated, 31 had carcinoid tumors, 10 each had insulinomas and gastrinomas, five had vipomas, nine had non-functioning islet cell tumors, three had glucagonomas, and one each had somatostatinoma and a possible cholecystokinin-secreting tumor (or CCKoma). Important lessons learned include: (1) the importance of preoperative tumor localization; (2) in multiple endocrine neoplasia, type I syndrome, the tumor found may not be the one responsible for the patient's symptoms; (3) solitary sporadic tumors secreting multiple peptides may mimic multiple tumors in multiple endocrine neoplasia, type I syndrome; (4) one needs to be prepared for the unexpected, such as the carcinoid crisis; (5) resection may sometimes be necessary even with advanced local disease; and (6) selected patients may benefit from pancreaticoduodenectomy.. These rare tumors are interesting in their clinical presentation and can be challenging in their treatment.

Topics: Adenoma, Islet Cell; Adult; Aged; Carcinoid Tumor; Cholecystokinin; Diagnosis, Differential; Digestive System Neoplasms; Female; Gastrinoma; Glucagonoma; Humans; Insulinoma; Male; Middle Aged; Multiple Endocrine Neoplasia; Neuroendocrine Tumors; Pancreatectomy; Retrospective Studies; Somatostatinoma; Vipoma