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Zollinger-Ellison syndrome

Definition:

Zollinger-Ellison syndrome is a condition caused by abnormal production of the hormone gastrin. A small tumor (gastinoma) in the pancreas or small intestine produces the high levels of gastrin in the blood.

Alternative Names:

Z-E syndrome; Gastrinoma

Causes, incidence, and risk factors:

Zollinger-Ellison syndrome is caused by tumors usually found in the head of the pancreas and the upper small bowel. These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid.

High stomach acid levels lead to multiple ulcers in the stomach and small bowel. Patients with Zollinger-Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel.

Gastrinomas occur as single tumors or as small, multiple tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that commonly spread to the liver and nearby lymph nodes. Nearly 25% of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients often have tumors of the pituitary gland (brain) and parathyroid (neck) glands in addition to tumors of the pancreas.

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