During a period of 13.5 years 17 patients with a
gastrinoma and an associated
Zollinger-Ellison syndrome were treated. In three patients (18%) the
gastrinoma was part of a
multiple endocrine neoplasia type I (MEN I). The median interval from the initial symptoms to the definite diagnosis was 5.0 years. During this interval seven patients (41%) underwent gastric surgery up to four times. The preoperative imaging studies localized the primary
tumor in only seven patients (41%). In five of six diagnostic
laparotomies the primary site of the
tumor was identified and proved by pathologic work-up. The
surgical procedures (n = 13) included five resections of the pancreas (3 x pancreatic head, 2 x left pancreatic resection), two duodenal resections, three enucleations of the
tumor and three palliative operations (hospital mortality: 0%). Following
laparotomy the
gastrinoma could be histologically proved in eleven of 17 patients (6 x pancreas, 4 x duodenum, 1 x in the hepatoduodenal ligament). The rate of metastatic spread as characteristic feature of
malignancy was 59%. After complete resection of the primary
tumor (n = 8) none of these patients died because of the
gastrinoma during the follow-up (median: 7.3 years). In the remaining patients three deaths were caused by the metastatic spread of the
gastrinoma. Considering the high rate of preceding operations, the high
malignancy rate and the excellent prognosis after RO-resection the diagnostic interval in patients with ZES is too long. Despite the modern radiographic imaging the exploratory
laparotomy is of high value in patients with ZES.