A personal experience with pancreatic and duodenal neuroendocrine tumors.J Am Coll Surg. 1999 Nov; 189(5):470-82.JA
Since the concept of hormones was proposed in 1901, numerous gastrointestinal hormones and neuroendocrine tumors that can produce these hormones have been identified. The most common tumors are gastrinomas and insulinomas.
During a 35-year experience, there were 82 neuroendocrine tumors, including 37 gastrinomas, 11 insulinomas, 16 nonfunctioning tumors, 11 gastrinomas suspected but not found, 3 tumors arising in lymph nodes, 1 somatostatinoma, 1 glucagonoma, and 2 amphicrine tumors. MEN I syndrome coexisted with three pancreatic gastrinomas, two pancreatic and duodenal gastrinomas, four suspected gastrinomas, one nonfunctioning tumor, two insulinomas, and no duodenal gastrinomas.
Of the nine patients with pancreatic gastrinoma without MEN I, three had lymph node, three had liver metastases, and one had both. The mean survival time was 4.8 years. Three patients with pancreatic gastrinoma and MEN I were alive at 2, 17, and 20 years, respectively. Of the 20 patients with duodenal gastrinoma, none had MEN I; 13 had lymph node metastases and 1 had liver metastases. The overall followup was 7.0 years. Ten patients were biochemically cured. Nonfunctioning tumors, with one exception, originated in the pancreas. Of the three gastrinomas potentially arising in lymph nodes, two, and possibly three, were cured by node removal. Eleven patients had an insulinoma. No patient had recurrence of hypoglycemia after removal of an insulinoma.
Patients with duodenal gastrinoma with lymph node metastases were curable, and cures were achieved occasionally after resection of liver metastases. Results of operation were similar for those with and without MEN I. MEN I and metastases were not contraindications to operation; instead, these patients should be operated on aggressively. Gastrinomas not found at operation were likely to be small duodenal gastrinomas. Gastrinomas can arise in a lymph node and can be cured by its removal. Parietal cell vagotomy is recommended after operation for gastrinomas in the event of residual tumor. With the exception of patients with MEN I or microadenomata, insulinomas were treated best by tumor enucleation. Otherwise, Whipple operation or distal pancreatectomy and enucleation of tumor in the remaining pancreas was indicated.