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Zollinger-Ellison Syndrome

(Z-E Syndrome; Gastrinoma)

Definition

Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver.
About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.
Gastric Ulcer
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Causes

Zollinger-Ellison syndrome is caused by gastrin-producing tumors, called gastrinomas. Gastrin is a hormone that stimulates the stomach to produce acid. Excess acid production may lead to ulcers in the stomach or duodenum.

Risk Factors

Factors that increase your chance of getting Zollinger-Ellison syndrome include:
  • Your or family members with MEN 1
  • History of endocrine disorders
  • Recurrent peptic ulcers

Symptoms

In many people, Zollinger-Ellison syndrome causes symptoms similar to an ulcer or gastroesophageal reflux disease (GERD).
Symptoms may include:
  • Abdominal pain
  • Heartburn
  • Nausea or vomiting
  • Fatigue
  • Diarrhea
  • Weight loss
  • Blood in the vomit or stool

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done.
Tests may include:
  • Blood tests—specifically to look for elevated levels of the hormone gastrin or evidence of MEN 1
  • Upper gastrointestinal endoscopy—a flexible tube with a light and camera is inserted down the throat and into the stomach and intestine to look for ulcers
Imaging tests may help your doctor to detect and localize the tumors. These tests may include:

Treatment

Talk with your doctor about the best treatment plan for you. Options include:

Surgical Removal of Tumor

Surgical removal of the gastrin-secreting tumors may be attempted. This may not be possible though if there are multiple tumors, or if cancer cells have spread to other parts of the body.

Medications for Ulcers

Zollinger-Ellison syndrome may be treated with:
  • Proton pump inhibitors
  • H-2 blockers
  • Somatostatin analogs to control tumor growth
  • Chemotherapy may be used in those who have rapidly growing tumors or for tumors that can't be removed

Prevention

There are no current guidelines to prevent Zollinger-Ellison syndrome. If you have a family history of Zollinger-Ellison syndrome or MEN 1, consider contacting a genetic counselor for screening.

RESOURCES

American Gastroenterological Association http://www.gastro.org

National Digestive Diseases Information Clearinghouse http://digestive.niddk.nih.gov

CANADIAN RESOURCES

The Canadian Association of Gastroenterology http://www.cag-acg.org

Health Canada http://www.hc-sc.gc.ca

References

Berna MJ, Hoffmann KM, et al. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National institutes of Health and comparison with 537 cases from the literature, evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine. 2006;85:331-364.

Campana D, Piscitelli L, et al. Zollinger-Ellison syndrome: diagnosis and therapy. Minerva Med. 2005;96:167-206.

Norton JA, Fraker DL, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999;341:635-644.

Norton JA, Jensen RT. Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison Syndrome. Ann Surg. 2004;240:757-773.

Tomassetti P, Salomone T, et al. Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. Drgus Aging. 2003;20:1019-1034.

Zollinger-Ellison syndrome. EBSCO DynaMed website. Available at: http://dynamed.ebscohost.com/about/about-us. Updated March 5, 2013. Accessed August 1, 2013.

Zollinger-Ellison syndrome. National Digestive Diseases Information Clearinghouse website. Available at: http://digestive.niddk.nih.gov/ddiseases/pubs/zollinger. Updated May 10, 2012. Accessed August 1, 2013.

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