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Zollinger-Ellison Syndrome

(Z-E Syndrome; Gastrinoma)

Definition

Zollinger-Ellison syndrome is a rare disorder where tumors form in the pancreas or duodenum (upper 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. They can spread to other parts of the body.

Causes

The exact cause of Zollinger-Ellison syndrome is not known. In some people, it is caused by a gene problem called multiple endocrine neoplasia type 1 (MEN 1).

Risk Factors

This problem is more common in men. Other things that raise the risk are:

  • Having MEN 1
  • A family history of MEN 1

Symptoms

Symptoms of Zollinger-Ellison syndrome may be:

  • Belly pain, bloating, and burping
  • Burning discomfort in the chest
  • Lack of energy
  • Nausea or vomiting
  • Loose stools (poop)
  • 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.

Upper endoscopy may be done—to look at the esophagus (food pipe), stomach, and duodenum.

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

Treatment

The goal is to control stomach acid and help the stomach heal. 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:

Gastrinoma. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/gastrinoma. Accessed July 27, 2021.

Gastrinoma. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/gastrointestinal-disorders/tumors-of-the-gastrointestinal-tract/gastrinoma. Accessed July 27, 2021.

Norton JA, Foster DS, et al. Gastrinomas: medical or surgical treatment. Endocrinol Metab Clin North Am. 2018;47(3):577-601.

Zollinger-Ellison syndrome. Cleveland Clinic website. Available at: https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome. Accessed July 27, 2021.

Zollinger-Ellison syndrome. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome. Accessed July 27, 2021.

Last reviewed July 2021 by EBSCO Medical Review Board Daniel A. Ostrovsky, MD  Last Updated: 7/27/2021