A staging system has been proposed by Ellison114 to develop predictive survival curves. Analysis of Z-E syndrome patients diagnosed at Ohio State University during a 40-year period indicated three determinants of survival: primary tumor size, presence of liver metastases, and complete resection of tumor. Factors with no effect on survival were age at diagnosis, sex, presence of lymph node metastases, and associated MEN. The expected 10-year survival for resected stage I tumors (primary < 2 cm, no liver metastases) was 94% to 96%; stage II (primary > 2 cm, no liver metastases), 86% to 91%; and stage III (liver metastases), 65% to 90%. When tumor was not resected, survival for stage I was 68% to 82%; stage II, 40% to 55%; and stage III, 7% to 50%.

A report by the NIH group analyzing 185 consecutive Z-E syndrome patients who were observed prospectively showed that survival was determined primarily by the presence of liver metastases at the time of admission.98 Liver metastases correlated with the size of the primary tumor and occurred more often with pancreatic than duodenal tumors. The 10-year survival rate was not significantly different between patients with gastrinoma found only in lymph nodes and patients with duodenal gastrinomas (100% and 94%, respectively); however, both groups had significantly better survival than the 59% survival rate of all patients with pancreatic gastrinomas. This report supports the concept that there are two distinct clinical forms of gastrinoma: benign and malignant.121

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