Zollinger-Ellison Syndrome

Zollinger-Ellison Syndrome, or ZES, is a rare digestive disorder in which tumors in the pancreas or duodenum produce large amounts of gastrin (a hormone). This abnormal production of too much gastrin causes the stomach to produce too much acid. As a result, ulcers can develop in the stomach and duodenum (first part of the small intestine)—a condition called peptic ulcer.

Causes

The cause of Zollinger-Ellison syndrome is not entirely clear. However, about 25 percent of ZES cases are associated with a condition called multiple endocrine neoplasia type 1, or MEN 1. MEN 1 is a rare inherited disorder that affects the endocrine glands including the pancreas.
Top of Page

Symptoms

Signs and symptoms of Zollinger-Ellison syndrome may be similar to those of a peptic ulcer. These may include:
 burning or gnawing pain in the abdomen

 diarrhea

 nausea and vomiting

 fatigue

 weakness

 weight loss

 bleeding in the digestive tract
Top of Page

Tests and Diagnosis

Diagnosis of Zollinger-Ellison syndrome is based on your medical history, signs and symptoms, and results of diagnostic tests, such as blood tests and endoscopy.
Blood tests. A sample of your blood is examined in a laboratory to measure levels of gastrin in your blood.

Endoscopy. To check for ulcers, your doctor may recommend a diagnostic procedure called endoscopy. This procedure involves looking at the lining of the stomach and duodenum through a lighted tube.
Top of Page

Treatment

Treatment of Zollinger-Ellison syndrome includes medications to reduce the production of too much stomach acid. First line of treatment are medicines called proton pump inhibitors, such as lansoprazole, omeprazole, pantoprazole, and rabeprazole. These medications help suppress acid production and promote healing.

Other treatment options may include surgery to treat peptic ulcers or to remove tumors in the pancreas or duodenum.
Top of Page

References:
National Cancer Institute Dictionary of Cancer Terms (n.d.). Definition of Zollinger-Ellison syndrome. National Cancer Institute (NCI), U.S. National Institutes of Health (NIH). Web URL: http://www.cancer.gov/templates/db_alpha.aspx?CdrlD=44262. Accessed: October 11, 2008

National Endocrine and Metabolic Diseases Information Service (March 2006). Multiple Endocrine Neoplasia Type 1 (NIH Publication No. 06—3048). National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH), Bethesda, MD. Web URL: http://www.endocrine.niddk.nih.gov/pubs/men1/men1.htm. Accessed: October 11, 2008

National Digestive Diseases Information Clearinghouse (September 2004). Zollinger-Ellison Syndrome (NIH Publication No. 04—4692). National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH), Bethesda, MD. Web URL: http://digestive.niddk.nih.gov/ddiseases/pubs/zollinger/index.htm. Accessed: October 11, 2008


Top of Page