Zollinger-Ellison syndrome was initially described as a non-beta islet cell tumor of the pancreas, with gastric acid hypersecretion, and severe peptic ulcer disease (PUD), which is less common now. The
tion superiorly, junction of the second and third portions of the duodenum inferiorly, and the junction of the neck and body of the pancreas medially.18 Gastrinomas may occur in the pancreas of patients with MEN type I, but, recently, it has become evident that gastrinomas are very frequently in the duodenum, regardless of their presence in the pancreas (see Figure 16-3). In addition, most patients have multiple, small submucosal lesions of the duodenum.19 Gastrinomas are usually malignant, and regional lymph node metastases are found in 50% of patients at the time of pancreatic surgery.11 They present in the fourth decade.1316 Patients with MEN type I or a family history should be screened for Zollinger-Ellison syndrome with at least one fasting serum gastrin level if they develop either clinical or laboratory features suggestive of the disease. The diagnosis of Zollinger-Ellison syndrome depends on proving autonomous gastrin secretion that does not respond to normal physiologic mechanisms. Therefore, a fasting serum gastrin is typically the initial test. Other helpful tests to establish the diagnosis are the
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