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Zollinger-Ellison Syndrome

What is Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome (ZES) is a rare digestive disorder. If you have ZES, you likely have 1 or more tumors in the first part of the small intestine, the pancreas, or both. These tumors are called gastrinomas. The tumors are sometimes cancerous and may spread to other areas of the body. They 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. In ZES, the ulcers are usually in the first part of the small intestine (the duodenum), and sometimes the second part (the jejunum).

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 small intestine.

Who is at risk 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 a 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, but often more severe and associated with diarrhea. Other symptoms include:

  • Nausea

  • Vomiting

  • Weight loss

  • Belly (abdominal) pain, 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 healthcare 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 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.

In some cases, you need surgery to remove tumors in your digestive tract. In the most severe cases, your healthcare provider may give you chemotherapy to destroy them. This might be when tumors have spread to other parts of your body.

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. The 10-year survival rate is very good, although a few people do get a more serious disease.

When should I call my healthcare provider?

ZES can sometimes be serious with severe complications if not treated. See your healthcare provider for a diagnosis if any of the symptoms last for more than a few days. Call your provider 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

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

  • 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 healthcare provider if you have questions.

Online Medical Reviewer: Jen Lehrer MD
Online Medical Reviewer: L Renee Watson MSN RN
Online Medical Reviewer: Marianne Fraser MSN RN
Date Last Reviewed: 4/1/2022
© 2000-2022 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.
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