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.
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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).
This problem is more common in men. Other things that raise the risk are:
Symptoms of Zollinger-Ellison syndrome may be:
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.
The goal is to control stomach acid and help the stomach heal. This can be done with medicines such as:
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.
There are no current guidelines to prevent Zollinger-Ellison syndrome.
American Gastroenterological Association
National Institute of Diabetes and Digestive and Kidney Diseases
Canadian Association of Gastroenterology
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