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Zollinger-Ellison Syndrome

(Z-E Syndrome; Gastrinoma)

Definition

Zollinger-Ellison syndrome is a rare disorder that happens when tumors form in the pancreas or duodenum (the upper part of the small intestine). The tumors cause the stomach to make a lot of acid. This can cause ulcers to form. The tumors may also be cancerous and spread to other parts of the body.

Gastric Ulcer
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Causes

The exact cause is not known. In some people, it is caused by a genetic problem called multiple endocrine neoplasia type 1 (MEN 1).

Risk Factors

This problem is more common in men. It is also more common in people who have MEN 1 and those who have people in their family with MEN 1.

Symptoms

Problems may be:

  • Belly pain and bloating
  • Heartburn
  • Burping
  • Lack of energy
  • Nausea or vomiting
  • Diarrhea
  • Weight loss
  • Lack of hunger
  • Blood in the vomit or stool

Diagnosis

The doctor will ask about your symptoms and health history. A physical exam will be done.

Blood tests will be done to look for high levels of the hormone gastrin. This can confirm the diagnosis.

More testing will be done to check for signs of MEN 1 and look for tumors.

Treatment

This problem is managed by controlling amount of acid made by the stomach. This can be done with medicines such as:

  • Proton pump inhibitors
  • H-2 blockers

Some people may need surgery to remove tumors. This may not be possible if there are many tumors or if cancer cells have spread to other parts of the body.

Prevention

There are no current guidelines to prevent Zollinger-Ellison syndrome.

RESOURCES:

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

National Institute of Diabetes and Digestive and Kidney Diseases
http://www.niddk.nih.gov

CANADIAN RESOURCES:

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

Health Canada
https://www.canada.ca

REFERENCES:

Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT. 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(6):331-364.

Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 2014;19(1):44-50.

Gastrinoma. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T115698/Gastrinoma. Updated May 22, 2017. Accessed February 12, 2018.

Gastrinoma. Merck Manual Professional Version website. Available at: http://www.merckmanuals.com/professional/gastrointestinal-disorders/tumors-of-the-gi-tract/gastrinoma. Updated October 2017. Accessed February 12, 2018.

Ito T, Igarashi H, Jensen RT. Zollinger-Ellison syndrome: recent advances and controversies. Curr Opin Gastroenterol. 2013;29(6):650-661.

Krampitz GW, Norton JA. Current management of the Zollinger-Ellison syndrome. Adv Surg. 2013;47:59-79.

Zollinger-Ellison syndrome. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/zollinger-ellison-syndrome/Pages/facts.aspx. Updated December 2013. Accessed February 12, 2018.

Last reviewed March 2018 by EBSCO Medical Review Board Daniel A. Ostrovsky, MD  Last Updated: 3/21/2016