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What Is Zollinger-Ellison Syndrome?

Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system. People who have ZES develop tumors known as gastrinomas in the pancreas and duodenum (the first section of the small intestine). The gastrinomas caused by ZES secrete the hormone gastrin. Because gastrin creates excessive stomach acid, patients with ZES can also develop stomach and duodenal ulcers.

What Are the Complications of Zollinger-Ellison Syndrome?

A person who has Zollinger-Ellison syndrome may have only one gastrinoma or may have several. Approximately 25% to 30% of ZES patients also have a genetic (inherited) disorder known as "multiple endocrine neoplasia type 1," which causes tumors in the pituitary and parathyroid glands.

Another complication of ZES is that in up to two-thirds of cases, single gastrinomas are malignant (cancerous). These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin.

What Are the Symptoms of Zollinger-Ellison Syndrome?

People who have Zollinger-Ellison syndrome don't always have symptoms. When symptoms do occur, they include:

How Is Zollinger-Ellison Syndrome Diagnosed?

If your doctor suspects that you have ZES, he or she will perform a blood test to look for high levels of gastrin (the hormone secreted by gastrinomas). They may also perform tests to measure how much acid your stomach is producing.

Your doctor may examine you for ulcers by performing an endoscopy. This procedure is done with a flexible, lighted tube (an endoscope) that looks at your esophagus, stomach and duodenum. This is often done with endoscopic ultrasound to see the tumor.

A CAT scan, a special type of X-ray that provides cross-sectional images of the body, may also be performed to locate the gastrinomas.

Despite these tests, gastrinomas can be difficult to find.

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