What is Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome (ZES) is a rare digestive disorder. If you have ZES, you likely have one or more tumors in the first part of the small intestine, the pancreas, or both. These tumors, called gastrinomas, release the hormone gastrin. This causes the stomach to release too much acid. Stomach acid is needed to break down food. But, too much acid can cause painful peptic ulcers inside the lining of your stomach and intestine. While gastrinoma tumors do cause health problems, they are typically not cancerous tumors.

What causes Zollinger-Ellison syndrome?

ZES is caused by tumors, called gastrinomas. The tumors cause the release of too much stomach acid. The extra acid can cause painful peptic ulcers inside the lining of your stomach and intestines.

What are the risk factors for Zollinger-Ellison syndrome?

Just about anyone can get ZES. But, some people with the condition may have a genetic problem known as multiple endocrine neoplasia type 1 (MEN1).  Children of adults with MEN1 are at an greater risk of getting the disease. ZES is more common in men, often those 30 to 50 years old.

What are the symptoms of Zollinger-Ellison syndrome?

The symptoms of ZES are similar to those of other ulcers. They include:

• Nausea
• Vomiting
• Weight loss
• Diarrhea
• Abdominal pain, sometimes burning in nature
• Severe heartburn (GERD or gastroesophageal reflux disease)
• Intestinal bleeding (such as black or tarry stool, or blood in the stool)

How is Zollinger-Ellison syndrome diagnosed?

If you have the symptoms associated with ulcers or ZES, your healthcare provider may measure your stomach acid levels. He or she may also give you a blood test to measure your level of the hormone gastrin, to see whether your body makes too much. Your healthcare provider may order imaging tests to look for tumors.

How is Zollinger-Ellison syndrome treated?

Medicines called proton pump inhibitors can help manage the extra stomach acid. These medicines help to stop ulcers from developing. They include esomeprazole, lansoprazole, pantoprazole, dexlansoprazole, rabeprazole, and omeprazole.

In some cases, surgery is needed to remove tumors in your digestive tract. In the most severe cases, including when tumors have spread to others parts of your body, your doctor may give you chemotherapy to destroy them.

What are the complications of Zollinger-Ellison syndrome?

In most people with ZES, tumors grow slowly and don’t spread quickly. If you can manage the ulcers, you can enjoy good quality of life. The 10-year survival rate is very good, although a few people do get more serious disease.

When should I call my healthcare provider?

ZES can sometimes be serious with severe complications, if left untreated. If any of the symptoms last for more than a few days, see your healthcare provider for a diagnosis. If you have any signs of intestinal bleeding, such as black or tarry stool, or blood in the stool, call your healthcare provider.

Key points

• Zollinger-Ellison syndrome is a rare digestive disorder that results in too much gastric acid.
• This excess gastric acid can cause peptic ulcers in your stomach and intestine.
• Symptoms include abdominal pain, nausea, vomiting, weight loss, and diarrhea.
• If left untreated, there can be serious complications.
• Treatment may include medicines and, sometimes, surgery.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.