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- Zollinger-Ellison syndrome (ZES) triad
- Markedly elevated gastric acid secretion
- Peptic ulcer disease
- A gastrinoma or non-β islet cell tumor of the pancreas or duodenal wall that produces gastrin
- Gastrinomas (at the time of diagnosis) may be single or multiple (1/2 to 2/3), large or small, benign or malignant (2/3), sporadic (70–75%) or associated with multiple endocrine neoplasia type 1 (MEN1) (25–30%).
- System(s) affected: endocrine/metabolic, gastrointestinal
- Synonym(s): Z-E syndrome; pancreatic ulcerogenic tumor syndrome; multiple endocrine neoplasia, partial; ulcerogenic islet cell tumor
- 1 to 3 per million per year in the United States
- Predominant age: middle age (30 to 65 years). Mean age of onset is 43 years; presents a decade earlier in patients with ZES/MEN1
- Predominant sex: male > female (1.3:1)
Aggressive cases have been reported in teenagers.
Rare, pregnancy alters medication choices and surgical timing.
Etiology and Pathophysiology
- Gastrinoma is equally distributed between the head of the pancreas and the first or second portion of the duodenum; if in the pancreas, the lesion is more likely to metastasize to the liver.
- Hypergastrinemia results in gastric mucosal hypertrophy and increased acid production. Increased acid production causes mucosal ulceration. Diarrhea and malabsorption are also common in ZES.
- Increasing number found in stomach wall, up to 8%; may be due to increased surveillance and/or increased proton pump inhibitor (PPI) use masking symptoms (1)[C]
- Also may be found rarely in the mesentery, peritoneum, spleen, skin, or mediastinum (possibly metastasis with primary not identified)
~25–30% of cases occur in association with the MEN1 syndrome—tumors of pancreas, pituitary, and parathyroid.
- Family history of ulcer disease
Screen first-degree relatives of patients with MEN1.
Commonly Associated Conditions
- Carcinoid tumors