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Zollinger-Ellison Syndrome

(Z-E Syndrome; Gastrinoma)

Definition

Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver.

About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.

Gastric Ulcer

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Causes    TOP

Zollinger-Ellison syndrome is caused by gastrin-producing tumors, called gastrinomas. Gastrin is a hormone that stimulates the stomach to produce acid. Excess acid production may lead to ulcers in the stomach or the first part of the small intestine called the duodenum.

Risk Factors    TOP

Factors that may increase your chance of Zollinger-Ellison syndrome include:

  • Your or family members with MEN 1
  • History of endocrine disorders
  • Recurrent peptic ulcers

Symptoms    TOP

In many people, Zollinger-Ellison syndrome causes symptoms similar to an ulcer or gastroesophageal reflux disease (GERD).

Symptoms may include:

  • Abdominal pain
  • Heartburn
  • Nausea or vomiting
  • Fatigue
  • Diarrhea
  • Weight loss
  • Blood in the vomit or stool

Diagnosis    TOP

Your doctor will ask about your symptoms and medical history. A physical exam will be done.

Tests may include:

  • Blood tests—specifically to look for elevated levels of the hormone gastrin or evidence of MEN 1
  • Upper gastrointestinal endoscopy—a flexible tube with a light and camera is inserted down the throat and into the stomach and intestine to look for ulcers

Imaging tests may help your doctor to detect and localize the tumors. These tests may include:

Treatment    TOP

Talk with your doctor about the best treatment plan for you. Options include:

Surgical Removal of Tumor

Surgical removal of the gastrin-secreting tumors may be attempted. This may not be possible though if there are multiple tumors, or if cancer cells have spread to other parts of the body.

Medications for Ulcers

Zollinger-Ellison syndrome may be treated with:

  • Proton pump inhibitors
  • H-2 blockers
  • Somatostatin analogs to control tumor growth
  • Chemotherapy may be used in those who have rapidly growing tumors or for tumors that can't be removed

Prevention    TOP

There are no current guidelines to prevent Zollinger-Ellison syndrome. If you have a family history of Zollinger-Ellison syndrome or MEN 1, consider contacting a genetic counselor for screening.

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

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 November 18, 2015. Accessed September 26, 2016.
Gastrinoma. Merck Manual Professional Version website. Available at:
...(Click grey area to select URL)
Updated July 2014. Accessed March 21, 2016.
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:
...(Click grey area to select URL)
Updated December 2013. Accessed March 21, 2016.
Last reviewed March 2017 by EBSCO Medical Review Board Michael Woods, MD, FAAP
Last Updated: 3/21/2016

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