What is Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome (ZES) is a rare digestive disorder that occurs when one or more tumors called gastrinomas located in your pancreas or duodenum cause your stomach to make too much acid.

What causes Zollinger-Ellison syndrome?

The exact cause of this disorder is unknown. Tumors called gastrinomas release large amounts of a hormone called gastrin, causing your stomach to make too much acid.

Who is at risk for Zollinger-Ellison syndrome?

Just about anyone can get ZES. But some people with the condition may have a rare genetic problem known as multiple endocrine neoplasia type 1 (MEN1). Children of adults with MEN1 are at a greater risk of getting the disease. ZES is more common in men, often those 20 to 50 years old.

What are the symptoms of Zollinger-Ellison syndrome?

The symptoms of ZES are similar to those of other ulcers, but they're often more severe and associated with diarrhea. Other symptoms include:

• Diarrhea, which may sometimes be the only symptom.

• Nausea.

• Vomiting.

• Weight loss.

• Belly (abdominal) pain, dull or sometimes burning in nature.

• Severe heartburn (gastroesophageal reflux disease or GERD).

• 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 health care provider may measure your stomach acid levels. They may also give you a blood test to measure your level of the hormone gastrin. This is to see whether your body makes too much. Other blood tests and an acid stimulation test are often used. Your 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 forming.

In some cases, you need surgery to remove tumors in your digestive tract. In the most severe cases, if the tumors are cancerous, your health care provider may give you chemotherapy.

What are possible 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 a good quality of life.

But the extra stomach acid caused by Zollinger-Ellison syndrome can lead to peptic ulcer disease or GERD. Without treatment, these diseases can lead to complications such as:

• Bleeding or perforation in the upper digestive tract.

• A blockage that can stop food from moving from your stomach into your duodenum.

• Esophagitis or esophageal stricture.

The tumors that cause Zollinger-Ellison syndrome are sometimes cancerous and may spread to other parts of your body. If the cancer spreads, it most often spreads to lymph nodes near the tumor and later to the liver and bones.

When should I call a doctor?

ZES can sometimes be serious with severe complications if not treated. See your health care provider for a diagnosis if any of the symptoms last for more than a few days. Seek emergency medical care if you have any signs of intestinal bleeding, such as black or tarry stool, blood in the stool, or vomiting blood.

Key points about Zollinger-Ellison syndrome

• Zollinger-Ellison syndrome is a rare digestive disorder that results in too much gastric acid.

• This extra gastric acid can cause peptic ulcers in your intestine.

• Symptoms include abdominal pain, nausea, vomiting, weight loss, and diarrhea.

• In most people, the ulcers can be managed if treatment suggestions are followed.

• If left untreated, there can be serious complications.

• Treatment may include medicines and sometimes surgery.

Next steps

Here are some tips to help you get the most from a visit to your health care provider:

• Know the reason for your visit and what you want to happen.

• 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 name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are and when they should be reported.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your health care provider if you have questions.