Zollinger-Ellison syndrome

Definition

What is Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system. 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.

How common is Zollinger-Ellison syndrome?

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. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Zollinger-Ellison syndrome?

The common symptoms of Zollinger-Ellison syndrome are:

• 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

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

You should contact your doctor if you have any of the following:

• Persistent, burning, aching or gnawing pain in your upper abdomen
• Nausea
• Vomiting
• Diarrhea

Causes

What causes Zollinger-Ellison syndrome?

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

What increases my risk for Zollinger-Ellison syndrome?

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.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

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.

Despite these tests, gastrinomas can be difficult to find.

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 lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix),

dexlansoprazole (Dexilant ), esomeprazole (Nexium), and rabeprazole (Aciphex), curb the production of stomach acid and allow the ulcers to heal.

Doctors may also prescribe medications known as H2-blockers, such as cimetidine (Tagamet), famotidine (Pepcid), and ranitidine (Zantac). However, these drugs 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. Up to 50% of patients who have surgery can be cured. For malignant tumors, radiation and chemotherapy may be offered.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Zollinger-Ellison syndrome?

Please discuss with your doctor for further information.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.