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Image of the MonthDiagnosis
Arch Surg. 2003;138:914.
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Answer: Zollinger-Ellison Syndrome
Figure 1. Hypertrophic folds within the body of the stomach, and a normal antrum.
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Figure 2. Duodenum and proximal jejunum revealing multiple ulcers.
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Gastrinomas are rare neuroendocrine tumors that secrete gastrin, producing severe peptic ulcer or even jejunal ulcer disease. Zollinger and Ellison1 were the first to attribute marked peptic ulcer disease in association with gastric acid hypersecretion and tumors of the pancreas. The discovery of gastrin in the 1960s helped further delineate the pathophysiology.2
Gastrinomas are the most common malignant pancreatic islet cell tumors. The incidence is rare, occurring in 0.1 to 3 million individuals per year.3 About 25% are associated with inherited familial disorders, such as multiple endocrine neoplasia type 1, and the remainder are sporadic. The incidence among patients with peptic ulcer disease is 0.1%; however, this approaches 1% in patients with recurrent disease.2 Sixty percent are malignant, of which 25% . . . [Full Text of this Article]
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