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- Zollinger-Ellison Syndrome 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 (1/2–2/3), large or small, benign or malignant (2/3), sporadic (70–75%) or associated with MEN1 (multiple endocrine neoplasia type 1) (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
- 1–3 per million per year
- Predominant age: Middle age (30–65 years)
- Predominant sex: Male > Female (1.3:1)
Very aggressive cases have been reported in teenagers.
Cases reported; influences medication choices and surgical timing
- Family history of ulcer disease
~25–30% of cases occur in association with the MEN1 syndrome.
Screen 1st-degree relatives of patients with MEN1.
- Gastrinoma is equally distributed between the head of the pancreas and the 1st or 2nd portion of the duodenum; if in the pancreas, the lesion is more likely to metastasize to the liver.
- Also may be found rarely in the mesentery, peritoneum, spleen, skin, or mediastinum (possibly metastasis with primary not identified)
Commonly Associated Conditions
- MEN1: Hyperparathyroidism, prolactinomas, other pituitary tumors
- Carcinoid tumors