Neuroendocrine gut neoplasms. Important lessons from uncommon tumors

Arch Surg. 1994 Sep;129(9):965-71; discussion 971-2. doi: 10.1001/archsurg.1994.01420330079015.


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.

Publication types

  • Case Reports

MeSH terms

  • Adenoma, Islet Cell / surgery
  • Adult
  • Aged
  • Carcinoid Tumor / surgery
  • Cholecystokinin / metabolism
  • Diagnosis, Differential
  • Digestive System Neoplasms / diagnosis
  • Digestive System Neoplasms / surgery*
  • Female
  • Gastrinoma / surgery
  • Glucagonoma / surgery
  • Humans
  • Insulinoma / surgery
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia / surgery
  • Neuroendocrine Tumors / diagnosis
  • Neuroendocrine Tumors / surgery*
  • Pancreatectomy / methods
  • Retrospective Studies
  • Somatostatinoma / surgery
  • Vipoma / surgery


  • Cholecystokinin