Zollinger-Ellison Syndrome
Condition
(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.
Treatment can help manage the condition.
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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
Zollinger-Ellison syndrome 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 symptoms and past health. 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 of Zollinger-Ellison syndrome.
Upper endoscopy may be done—to look at the food pipe (esophagus), 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.
American Gastroenterological Association
National Institute of Diabetes and Digestive and Kidney Diseases
CANADIAN RESOURCES:
Canadian Association of Gastroenterology
Health Canada
American Gastroenterological Association
National Institute of Diabetes and Digestive and Kidney Diseases
CANADIAN RESOURCES:
Canadian Association of Gastroenterology
Health Canada
References
- Gastrinoma. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/gastrinoma.
- Gastrinoma. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/gastrointestinal-disorders/tumors-of-the-gastrointestinal-tract/gastrinoma.
- 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.
- 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.
Contributors
- Daniel A. Ostrovsky, MD
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