Neuroendocrine gut neoplasms. Important lessons from uncommon tumors.

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.
SETTING: University hospitals with tertiary care referral practice.
INTERVENTIONS: The main intervention was abdominal exploration in 43 patients, with resection of the primary tumor in 39 and of hepatic metastases in four. MAIN OUTCOME MEASURES: To describe the tumors seen and to identify major lessons learned.
RESULTS: 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.
CONCLUSIONS: These rare tumors are interesting in their clinical presentation and can be challenging in their treatment.