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
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:
- Weight loss
- 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.
- Zollinger-Ellison syndrome is a rare digestive disorder that results in too much gastric
- 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.
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
- 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
- Know how you can contact your provider if you have questions.