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A.D.A.M. Medical Encyclopedia [Internet]. Atlanta (GA): A.D.A.M.; 2013.

A.D.A.M. Medical Encyclopedia.

Zollinger-Ellison syndrome

Z-E syndrome; Gastrinoma

Last reviewed: October 8, 2012.

Zollinger-Ellison syndrome is a condition in which there is increased production of the hormone gastrin. Most of the time, a small tumor (gastrinoma) in the pancreas or small intestine produces the extra gastrin in the blood.

Causes

Zollinger-Ellison syndrome is caused by tumors. These growths are most often found in the head of the pancreas and the upper small intestine. The tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause production of too much stomach acid.

Gastrinomas occur as single tumors or several tumors. About one-half to two-thirds of single gastrinomas are cancerous (malignant) tumors. These tumors often spread to the liver and nearby lymph nodes.

Many patients with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). People with this condition often have tumors of the pituitary gland (brain) and parathyroid gland (neck), as well as tumors of the pancreas.

Exams and Tests

Signs include ulcers in the stomach and small intestine.

Tests include:

Treatment

Drugs called proton pump inhibitors (omeprazole, lansoprazole, and others) are now the first choice for treating this condition. These drugs reduce acid production by the stomach, and help the ulcers in the stomach and small intestine heal. They also relieve abdominal pain and diarrhea.

Surgery to remove a single gastrinoma may be done if the tumors have not spread to other organs. Surgery on the stomach (gastrectomy) to control acid production is rarely needed.

Outlook (Prognosis)

The cure rate is low even with early detection and surgery to remove the tumor. However, gastrinomas grow slowly, and patients may live for many years after the tumor is discovered. Acid-suppressing medicines work well to control the symptoms of too much acid production.

Possible Complications

When to Contact a Medical Professional

Call your health care provider if you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.

References

  1. Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 32.

Review Date: 10/8/2012.

Reviewed by: George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, and Stephanie Slon.

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The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

What works?

  • Additional bedtime medication for the control of night‐time acid reflux from the stomach.Additional bedtime medication for the control of night‐time acid reflux from the stomach.
    The inhibition of gastric acid secretion is an accepted treatment for diseases related to reflux of acid from the stomach. Some types of antacids, known as proton pump inhibitors (PPI), are considered to be the most effective medical treatment for people patients with acid‐related diseases such as peptic ulcer, gastro‐oesophageal reflux disease (GERD) and Zollinger‐Ellison syndrome, but they may not reduce gastric acid secretion sufficiently to prevent night‐time acid reflux symptoms. H2‐receptor antagonists (H2RAs) have also been used for the treatment of acid‐related diseases for more than a decade and might help to control night‐time acid reflux, if taken at bedtime along with a high dose of PPI. The results show that additional bedtime H2RAs can decrease the night‐time gastric acid breakthrough, but we believe that additional bedtime H2RAs to PPI should only be used as treatment an intervention in clinical trials until further evidence has been found.
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  • Endocrine glands.

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