What Is Zollinger-Ellison Syndrome?

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Zollinger-Ellison syndrome (ZES) is a rare disorder in which tumors, called gastrinomas, develop in the small intestine (duodenum) or the pancreas. These tumors secrete a hormone called gastrin, which then causes excess amounts of stomach acid to be produced.

The excess stomach acid can cause the development of painful ulcers in the gastrointestinal tract. This rare condition is found in approximately one in one million people and was first identified in 1955 by Drs. Robert Zollinger and Edward Ellison in Columbus, Ohio.

This article will review the symptoms found with ZES, as well as why it happens and how it is diagnosed and treated.

Man holding his stomach in pain

Diy13 / Getty Images

Symptoms

The symptoms associated with ZES are caused by the presence of high levels of stomach acid, high levels of gastrin, or due to ulcers that form because of the disorder.

These symptoms can include:

  • Dull pain in the upper abdomen or chest
  • Burning pain in the upper abdomen or chest
  • Diarrhea
  • Bloating
  • Unexplained weight loss
  • Nausea and vomiting
  • Poor appetite

Causes

During the normal digestive process, food is chewed up and swallowed. As it enters the stomach, acid is present to help start to break down the food particles.

After its time in the stomach, the food and acid then move to the duodenum, the first part of the small intestine. In the duodenum, food continues to break down.

The pancreas is a nearby organ that assists with digestion. It releases insulin, which helps use sugar in the blood for energy. The pancreas also produces and releases other enzymes (compounds that aid in the digestion of food), which are emptied into the duodenum. 

With ZES, tumors—called gastrinomas—form in the duodenum and pancreas. These tumors then release large amounts of a hormone called gastrin, which tells the stomach to produce more acid than would typically be required for food digestion.

This excess stomach acid can irritate the lining of the duodenum, stomach, and esophagus, and can cause symptoms associated with the disease.

The exact cause of why gastrinomas form is often not known, but there is a link between gastrinomas and a genetic disorder called multiple endocrine neoplasia type 1 (MEN1).

About 25% of people with ZES also have MEN1. These tumors are often slow growing and benign, but may become cancerous.

Diagnosis

Diagnosing ZES is a multi-step process that may require many tests and imaging. 

History and Physical Exam

The healthcare provider will often start with a review of the patient’s history and the symptoms they are experiencing. A physical exam may also be done to evaluate the cause of the symptoms being experienced. 

Blood Test

A blood test to evaluate the amount of gastrin in the blood may be done. This level will be elevated, often 10 times higher than normal, in someone with ZES.

Upper Endoscopy

During an upper gastrointestinal endoscopy, a scope with a camera on the end is inserted into the mouth and moved down the esophagus and into the stomach and duodenum.

This allows a gastroenterologist to visualize the digestive tract, and evaluate for any tumors or ulcers that may be present. If needed, biopsies of any suspicious areas can be taken to be evaluated under the microscope. This exam is often done under anesthesia.

Imaging Tests

  • Computed tomography (CT): A CT scan is a radiological test that can take pictures of the body, and may be useful to look at the esophagus, stomach, small intestine, and pancreas. A special dye may be injected during the exam, and a liquid contrast agent may be ingested to help image these areas better.
  • Magnetic resonance imaging (MRI): An MRI uses a specialized machine with magnets to obtain images of the body without radiation. Sometimes an MRI can give a more detailed picture of the body system that is needed for a diagnosis. 
  • Endoscopic ultrasound: This procedure uses a special scope and ultrasound to navigate through the digestive system to be able to see the pancreas. It is often done under anesthesia. 
  • Octreotide scan: During this test, a special tracer is injected through an IV to be taken up by tumor cells. A special camera, called a gamma camera, is used to see if any cells took up the tracer and to determine their location. 

Stomach Acid Test

During a test for stomach acid, a tube is placed through the nose and into the stomach. This tube can be used to measure the amount of acid present in the stomach. Elevated levels are usually found in someone with ZES. 

Treatment

Multiple options exist to treat people with ZES.

Surgery

Sometimes surgery may be done to remove gastrinomas. This is not always possible, as oftentimes the gastrinomas are very small and difficult to see on imaging studies. There is also the possibility that gastrinomas can grow back, although typically very slowly, after surgical removal.

Medications

Medications can be prescribed to reduce the amount of stomach acid being put into the stomach. These medications—called proton pump inhibitors—include omeprazole, pantoprazole, and esomeprazole.

Higher-than-usual doses of these medications may be required in people with ZES. Decreasing the stomach acid can allow the tissue of the stomach and small intestine to heal, and can help treat ulcers. 

Chemotherapy

If the tumors in the duodenum or pancreas become cancerous, chemotherapy may need to be given.

Prognosis

It can be difficult to cure ZES, as the only possible curative therapy is surgery and it can be difficult to find and remove all of the tumors. However, gastrinomas tend to be very slow growing, and people can live for many years with this disease.

A Word From Verywell

A diagnosis of ZES can be concerning, but it is usually a slow-growing disease and is not life-threatening if treated early. If you notice changes in the stool or the development of burning or gnawing pain in the abdomen or chest, seek medical treatment to evaluate these symptoms.

People with ZES can live long lives following diagnosis. Managing symptoms is an important part of extending your lifespan.

Frequently Asked Questions

Is surgery the only way to cure Zollinger-Ellison syndrome?

Yes, surgery is the only way to cure Zollinger-Ellison syndrome. It is estimated that approximately 20% to 30% of ZES cases can be cured.

What are telltale symptoms of ZES?

The telltale symptoms of ZES include a dull or burning pain in the abdomen or chest, diarrhea, nausea, vomiting, and weight loss. 

What is the survival rate of Zollinger-Ellison syndrome?

The survival rate can vary from person to person living with ZES. Most people can live a long life. Long-term survival is lowest if the gastrinomas become cancerous and spread to other areas of the body.

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4 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
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  2. Kaplan EL, Tanaka R, Ito K, Younes N, Friesen SR. The discovery of the Zollinger-Ellison syndromeJ Hepatobiliary Pancreat Sci. 1994;1(5):509-516. doi:10.1007/BF01211912

  3. National Organization for Rare Disorders. Zollinger-Ellison syndrome.

  4. MedlinePlus. Zollinger-Ellison syndrome. Updated September 1, 2021.