Overview

Zollinger-Ellison syndrome is a rare condition in which one or more tumors form in your pancreas or the upper part of your small intestine (duodenum). These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid then leads to peptic ulcers, as well as to diarrhea and other symptoms.

Zollinger-Ellison syndrome (ZES) is rare. The disease may occur at any time in life, but people are usually diagnosed between ages 20 and 50. Medications to reduce stomach acid and heal the ulcers is the usual treatment for Zollinger-Ellison syndrome.

Symptoms

Signs and symptoms of Zollinger-Ellison syndrome may include:

  • Abdominal pain
  • Diarrhea
  • Burning, aching, gnawing or discomfort in your upper abdomen
  • Acid reflux and heartburn
  • Nausea and vomiting
  • Bleeding in your digestive tract
  • Unintended weight loss
  • Decreased appetite

When to see a doctor

See your doctor if you have a persistent, burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea.

Tell your doctor if you've used over-the-counter acid-reducing medications such as omeprazole (Prilosec), cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time. These medications may mask your symptoms, which could delay your diagnosis.

Causes

The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.

Your pancreas sits behind and below your stomach. It produces enzymes that are essential to digesting food. The pancreas also produces several hormones including insulin, a hormone that helps to control your blood glucose.

Digestive juices from the pancreas, liver and gallbladder mix in the duodenum, the part of the small intestine next to your stomach. This is where digestion reaches its peak.

The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of gastrin, which in turn causes the stomach to produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.

Besides causing excess acid production, the tumors are cancerous (malignant). Although the tumors tend to grow slowly, the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.

Association with MEN 1

Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). People with MEN 1 also have tumors in the parathyroid glands and may have tumors in their pituitary glands.

About 25 percent of people who have gastrinomas have them as part of MEN 1. They may have also have tumors in the pancreas and other organs.

Risk factors

If you have a blood relative, such as a sibling or parent, with MEN 1, it's more likely that you'll have Zollinger-Ellison syndrome.

Oct. 17, 2015
References
  1. Greenberger NJ, et al. Zollinger-Ellison syndrome. Current Diagnosis & Treatment: Gastroenterology, Hepatology & Endoscopy. 2nd ed. New York, N.Y.: The McGraw-Hill Companies. http://www.accessmedicine.com. Accessed Aug. 2, 2015.
  2. Zollinger-Ellison syndrome. National Institute of Diabetes and Digestive and Kidney Diseases. http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/zollinger-ellison-syndrome/Pages/facts.aspx. Accessed Aug. 2, 2015.
  3. Cameron JL, et al, eds. Zollinger-Ellison syndrome. In: Current Surgical Therapy. 11th ed. Philadelphia, Pa.; Saunders Elsevier: 2014. http://www.accessmedicine.com. Accessed Aug. 2, 2015.
  4. Goldfinger SE. Zollinger-Ellison syndrome (gastrinoma): Clinical manifestations and diagnosis. http://www.uptodate.com. Accessed Aug. 2, 2015.
  5. Krampitz GW, et al. Current management of the Zollinger-Ellison syndrome. Advances in Surgery. 2013;47:59.
  6. FDA Drug Safety Communication: Possible increased risk of fractures of the hip, wrist, and spine with the use of proton pump inhibitors. http://www.fda.gov/Drugs/DrugSafety/PostmarketDrugSafetyInformationforPatientsandProviders/ucm213206.htm. Accessed Aug. 5, 2015.

Zollinger-Ellison syndrome