Abstract
In 1947, a patient with metastatic islet cell tumor was treated for intractable ulcer disease at the University of Chicago Medical Center. Eight years later, in retrospect, it was recognized that he and another patient had the Zollinger-Ellison syndrome (ZE). From 1947 until the present, 30 patients with the ZE syndrome have been treated at this institution. Twenty-one (70%) were male and 9 (30%) were female. Their ages ranged from 24 to 76 years. Most (79%) had abdominal pain, however, melena (42%), hematemesis (33%), and severe diarrhea (35%) were prominent as well. Symptoms were present for a mean of 5.8 years before diagnosis. Over their entire clinical course, duodenal ulcers occurred in 96% of patients, gastric ulcers in 24%, jejunal ulcers in 29%, esophageal ulcers in 6%, and stomal ulcerations in 58%. Eleven (38%) of all gastrinomas were proved to occur in the duodenum; 10 (34%) were pancreatic in origin, including 3 with the MEN I syndrome; 3 (10%) were extrapancreatic and extraduodenal in origin, and no tumor was found in 5 (17%). Each of the 3 patients with MEN I developed a proven pancreatic islet cell carcinoma with metastases as well as hyperparathyroidism and a pituitary lesion. Of 27 patients who were explored for gastrinoma, tumor was found in 20 (74%). Excluding patients with MEN who had multiple lesions throughout the pancreas, all tumors were found in the "gastrinoma triangle." Total gastrectomy was performed in 10 (37%) of 27 of all patients who were explored, in 5 (71%) of 7 when no tumor was found, and in only 5 (25%) of 20 when tumor was present. Operative mortality was 15% (4 of 27), but no death has occurred since 1974. Long-term survival has followed both tumor resection or total gastrectomy in selected individuals (including 1 patient with known multiple liver metastases who is alive 18 years after liver biopsy and total gastrectomy); however, since malignant gastrinomas were present in 46% of all patients (or 57% in whom tumor was found) and since local metastases can sometimes be removed, we favor an aggressive approach to localization and resection when liver metastases or other distant metastases are not found. Duodenal gastrinomas are particularly favorable for resection for cure. They were malignant in only 36% and their metastases were nodal in each of 4 cases. The major problem is finding them since they are often small and "occult." All patients with ZE should have duodenoscopy preoperatively and, at operation, a wide duodenotomy with eversion of the duodenal mucosa and careful inspection and palpation should be performed. Above all, surgeons should realize that at least 38% of all gastrinomas may be duodenal in origin. Finally, since the early 1970's when serum gastrin measurements became available, 43% of our patients who were explored have been returned to basal eugastrinemia. © 1990 Société Internationale de Chirurgie.