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Zollinger-Ellison syndrome

Zollinger-Ellison syndrome is a condition in which there is increased production of the hormone gastrin. Most of the time, a small tumor (gastrinoma) in the pancreas or small intestine produces the extra gastrin in the blood.

Alternative Names

Z-E syndrome; Gastrinoma

Causes

Zollinger-Ellison syndrome is caused by tumors. These growths are most often found in the head of the pancreas and the upper small intestine. The tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause production of too much stomach acid.

Gastrinomas occur as single tumors or several tumors. About one-half to two-thirds of single gastrinomas are cancerous (malignant) tumors. These tumors often spread to the liver and nearby lymph nodes.

Many patients with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). People with this condition often have tumors of the pituitary gland (brain) and parathyroid gland (neck), as well as tumors of the pancreas.

Symptoms

Exams and Tests

Signs include ulcers in the stomach and small intestine.

Tests include:

Treatment

Drugs called proton pump inhibitors (omeprazole, lansoprazole, and others) are now the first choice for treating this condition. These drugs reduce acid production by the stomach, and help the ulcers in the stomach and small intestine heal. They also relieve abdominal pain and diarrhea.

Surgery to remove a single gastrinoma may be done if the tumors have not spread to other organs. Surgery on the stomach (gastrectomy) to control acid production is rarely needed.

Outlook (Prognosis)

The cure rate is low even with early detection and surgery to remove the tumor. However, gastrinomas grow slowly, and patients may live for many years after the tumor is discovered. Acid-suppressing medicines work well to control the symptoms of too much acid production.

Possible Complications

  • Failure to locate the tumor during surgery
  • Intestinal bleeding or hole (perforation) from ulcers in the stomach or duodenum
  • Severe diarrhea and weight loss
  • Spread of the tumor to other organs

When to Contact a Medical Professional

Call your health care provider if you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.

References

Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 32.

Updated: 10/8/2012

George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, and Stephanie Slon.


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