Pancreatic Islet Cell Tumors

Pancreatic or duodenal neuroendocrine tumors in MEN type I occur most frequently in the fourth or fifth decade. Greater than 95% of those who develop enteropancreatic neuroendocrine tumors already have hyperparathyroidism.13 However, Zollinger-Ellison syndrome may be the first manifestation in a small population of patients.14 The clinical presentation usually depends on the increased hormone levels; therefore, patients may present early when tumors are small, for example, with duodenal gastrinomas that are unde-tectable on preoperative imaging but that cause significant ulcer disease, esophageal reflux symptoms, and diarrhea (Figure 16-3). Symptoms of local enlargement or infiltration including back pain and abdominal mass, left-sided portal hypertension, jaundice, or metastatic disease (cachexia, hepatosplenomegaly) may rarely be present at presentation, but more frequently in older patients with nonfunctional tumors. Nonfunctional neuroendocrine tumors, of which three-

Table 16-1. MULTIPLE ENDOCRINE NEOPLASIA TYPE I CLINICAL DISEASE EXPRESSION
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