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Zollinger-Ellison Syndrome (ZES) is characterized by ulcer disease and dyspepsia due to inappropriate secretion of gastrin by neuroendocrine tumors called gastrinomas. Gastrinomas represent only a small fraction of pancreatic and duodenal tumors. They may occur sporadically (75%) or in association with genetic endocrine disorders such as MEN I (25%), also known as Wermer syndrome.1Differences in the pathogenesis of gastrinomas in these 2 subtypes have implications for presentation, treatment, and survival.
Patients with MEN I often present with ZES at a younger age, typically in the fourth decade of life.1,2The diagnosis is based on elevated gastrin levels in the presence of gastric hyperacidity. Secretin stimulation of gastrin levels may be required to diagnose ZES. The severity of symptoms and degree of gastrin level elevation does not differ by tumor location or by the subtype of disease.
Image of the Month—Diagnosis. Arch Surg. 2009;144(4):378. doi:10.1001/archsurg.2009.7-b
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