Zollinger-Ellison Syndrome Symptoms

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Zollinger-Ellison Syndrome (ZES) is a rare disorder that causes one or more tumors to form in the pancreas or the upper part of the small intestine called the duodenum. It can also cause ulcers to develop in the stomach and the duodenum.

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The tumors are called gastrinomas, and they secrete a large amount of the hormone gastrin. This then causes excessive production of stomach acid, which can lead to peptic ulcers. The tumors are cancerous in 50 percent of the cases.

The ulcers caused by ZES are less responsive to treatment than ordinary peptic ulcers. What causes people with ZES to develop tumors is unknown, but approximately 25% of ZES cases are associated with a genetic disorder called multiple endocrine neoplasia.

Zollinger-Ellison syndrome is rare, and though it may occur at any age, people between the ages of 30 and 60 are more likely to develop it. Also, of all the people who suffer from a peptic ulcer, only a tiny percentage of those people will have Zollinger-Ellison.

Zollinger-Ellison Syndrome Symptoms

The symptoms of ZES include:

  • Gnawing, burning pain in the abdomen: This pain is usually located in the area between the breastbone and the navel.
  • The sensation of pressure, bloating, or fullness: This pain usually develops 30 to 90 minutes after a meal and is often relieved by antacids.
  • Pain or burning sensation in the abdomen that travels up toward the throat: This is caused by heartburn, or gastroesophageal reflux, and occurs when stomach contents back up into the esophagus.
  • Vomiting: The vomit may contain blood or resemble coffee grounds.
  • Diarrhea: Stools may be foul-smelling.
  • Black, tarry stools: Blood in the stools will turn them dark red or black, and make them tarry or sticky.
  • Nausea
  • Fatigue
  • Weakness
  • Weight loss


Zollinger-Ellison syndrome is caused by a tumor or tumors in the pancreas and the duodenum. These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid. This increase in acidity can lead to the development of peptic ulcers in the stomach and duodenum.


A blood test is performed to see whether there is an increased gastrin level in the blood. An elevated level of gastrin may indicate tumors in the pancreas or duodenum.

Barium X-Ray

The patient drinks a liquid that contains barium, which will coat the walls of the esophagus, stomach, and duodenum. X-rays are then taken. The healthcare provider will then view the X-rays, looking for signs of ulcers.

Upper Endoscopy

The healthcare provider examines the inside of the esophagus, stomach, and duodenum with an instrument called an endoscope, a thin, flexible, lighted tube with a lens. The endoscope is inserted through the mouth and down the throat, and into the stomach and duodenum.

The practitioner can look for ulcers, and can also remove a tissue sample, called a biopsy, for examination in the laboratory to identify if there is the presence of gastrin-producing tumors.

Imaging Techniques

A healthcare provider may use a computerized tomography (CT) scan, a magnetic resonance imaging (MRI) scan, and ultrasound, or a nuclear scan in an effort to pinpoint where tumors may be located:

  • Computed tomography (CT): This diagnostic test that uses X-rays aided by computer technology. The X-ray beams are taken from many different angles to create cross-sectional images of the patient's body. Then a computer assembles these images into a three-dimensional picture that can display organs, bones, and tissues in great detail.
  • Magnetic resonance imaging (MRI): With an MRI scan, magnetic signals are used rather than X-rays to create images of the human body. These images show the differences between types of tissues.
  • Ultrasound: An ultrasound sends out high-frequency sound waves that go into the area being examined and bounce back when they hit an organ. This is processed by a computer, which produces a map of the area being scanned.
  • Nuclear scan: With a nuclear scan, a radioactive substance is introduced into the body that permits a gamma camera to detect tumors.


In 50% of cases of Zollinger-Ellison, the tumors are cancerous (malignant). If the tumors are cancerous, there is a risk that cancer will spread to the liver, the lymph nodes near the pancreas and small intestine.

Other complications of Zollinger-Ellison Syndrome:

  • Severe ulcers that may lead to the development of a hole in the bowel
  • Blood loss
  • Chronic diarrhea
  • Weight loss


The treatment of Zollinger-Ellison syndrome focuses on two areas—treating the tumors and treating the ulcers.

Surgery is often performed if there is only one tumor. If tumors are in the liver, a surgeon will remove as much of a liver tumor as possible (debulking).

When surgery on tumors isn't possible, other treatments are used:

  • Attempting to destroy the tumor by cutting off the blood supply (embolization).
  • Attempting to destroy cancer cells by using an electric current (radio-frequency ablation).
  • Injecting drugs into the tumor to relieve cancer symptoms.
  • Using chemotherapy to try to slow tumor growth.

Medications for Ulcers

Proton pump inhibitors are the most effective medications for Zollinger-Ellison syndrome. Proton pump inhibitors are quite powerful and suppress acid production and promote healing. Examples of proton pump inhibitors include Prilosec, Prevacid, Nexium, Aciphex, and Protonix.

Acid blockers, also called histamine (H-2) blockers, reduce the amount of hydrochloric acid released into the digestive tract. This helps to relieve ulcer pain and encourage healing.

Acid blockers work by keeping histamine from reaching histamine receptors. Histamine receptors signal acid-secreting cells in the stomach to release hydrochloric acid. Examples of acid blockers include Tagamet, Pepcid, and Axid.

Acid blockers don't work as well as proton pump inhibitors do, and may not be prescribed. For those people who do use acid blockers, they need high, frequent doses for them to be effective.


As stated above, in 50% of cases of Zollinger-Ellison, the tumors are cancerous. Early diagnosis and surgical invention can achieve a cure rate of only 20% to 25%.

However, gastrinomas are slow-growing, and patients may live for many years after a tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of acid overproduction.

A Word From Verywell

For a diagnosis, you need to see your healthcare provider. At this time all aspects of this condition, along with treatment options and ongoing care and monitoring of the condition, should be discussed with your practitioner. After diagnosis, if you should experience and new or worsening symptoms, these should be reported to your healthcare provider.

2 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.
  1. National Organization for Rare Disorders (NORD). Zollinger-Ellison syndrome.

  2.  National Institute of Diabetes and Digestive and Kidney Diseases. Zollinger-Ellison syndrome. December 2013

By Sharon Gillson
 Sharon Gillson is a writer living with and covering GERD and other digestive issues.