What Is Zollinger-Ellison Syndrome?

Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system (stomach, intestines, etc.). People who have ZES develop tumors known as gastrinomas in the pancreas and duodenum (the first section of the small intestine). The gastrinomas caused by ZES secrete the hormone gastrin. Because gastrin creates excessive stomach acid, patients with ZES can also develop stomach and duodenal ulcers.

What Are the Complications of Zollinger-Ellison Syndrome?

A person who has Zollinger-Ellison syndrome may have only one gastrinoma or may have several. Approximately one-quarter of ZES patients also have a genetic (inherited) disorder known as "multiple endocrine neoplasia type 1," which causes tumors in the pituitary and parathyroid glands.

Another complication of ZES is that in up to two-thirds of cases, gastrinomas are malignant (cancerous). These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin.

What Are the Symptoms of Zollinger-Ellison Syndrome?

People who have Zollinger-Ellison syndrome don't always have symptoms. When symptoms do occur, they include:

• Burning pain in the abdomen
• Nausea
• Diarrhea
• Vomiting
• Bleeding
• Weakness
• Fatigue

How Is Zollinger-Ellison Syndrome Diagnosed?

If your doctor suspects that you have ZES, he or she will perform a blood test to look for high levels of gastrin (the hormone secreted by gastrinomas). They may also perform tests to measure how much acid your stomach is producing.

Your doctor may examine you for ulcers by performing an endoscopy. This procedure is done with a flexible, lighted tube (an endoscope) that looks at your esophagus, stomach and duodenum. This is often done with endoscopic ultrasound to see the tumor.

A CAT scan, a special type of x-ray that provides cross-sectional images of the body, may also be performed to locate the gastrinomas.

How Is Zollinger-Ellison Syndrome Treated?

ZES is treated by reducing the amount of acid your stomach produces. Medications called proton pump inhibitors are usually prescribed. These drugs, which include Prevacid, Prilosec, Protonix, Nexium, and Aciphex, curb the production of stomach acid and allow the ulcers to heal.

Doctors may also prescribe medications known as H2-blockers, such as Tagamet, Pepcid, and Zantac. However, these medications don't work as well to reduce stomach acid.

In some cases, a patient may need surgery for the peptic ulcer or to remove the gastrinoma. However, only about 20-25% of patients who have surgery are cured. For malignant tumors, radiation and chemotherapy may be offered.

What Is the Outlook for People with Zollinger-Ellison Syndrome?

Gastrinomas tend to grow slowly, and are not always malignant. Approximately two-thirds of all patients will survive five years. The five-year survival rate depends on whether the tumors have spread. If they have not spread to the liver, the five-year survival rate is approximately 90%. The percentage drops to 20% if the tumors have spread to the liver.

Treatment Follow-up for ZES

If you have been treated for ZES, you should see your doctor on a regular basis to determine if the gastrinomas recur.

Reviewed by The Cleveland Clinic Department of Gastroenterology.