Zollinger-Ellison Syndrome (ZES)

What is Zollinger-Ellison Syndrome?

Zollinger-Ellison Syndrome is a very rare disease that affects the gastrointestinal system. The syndrome is characterized by the presence of a single or multiple gastrinomas.

A gastrinoma is a generally malignant tumor located in the pancreas or in the upper part of the intestine (duodenum) that secretes excessive amounts of gastrin. In turn, this peptide hormone stimulates an exaggerated production of gastric acid (HCI) that creates ulcers in the stomach, pancreas and small intestine.

The disease can affect individuals at any time but it is mostly diagnosed between the age of 20 and 50.

Circa one third of the patients with ZESĀ  also have a genetic disorder known as multiple endocrine neoplasia type 1 which can cause tumors in glands (specifically in the pituary and parathyroid glands).

Gastrinomas can spread throughout the body and reach the liver, lymph nodes, bones, spleen, skin.

There are no known causes and risk factors connected to the Zollinger-Ellison Syndrome apart from its correlation with multiple endocrine neoplasia 1.

What are the Symptoms of Zollinger-Ellison Syndrome?

ZES can sometimes be asymptomatic. If the symptoms are present, these might include:

  • Nausea/Vomiting
  • Diarrhea
  • Acute pain in the abdominal area
  • Stomach bleeding
  • Burning sensation in the upper abdomen
  • Heartburn
  • Sudden weight loss
  • Weakness/fatigue and poor appetite

The diagnosis of ZES can be confirmed by high levels of gastrin found in a blood sample or high levels of acid in the stomach.

Causes Zollinger-Ellison Syndrome

Zollinger-Ellison Syndrome (ZES) is a condition where tumors form inside of the pancreas or the upper portion of the small intestines. These tumors produce gastrinomas, which secrete large amounts of the hormone gastrin, which produce excess acid in the stomach. This excessive acid leads to peptic ulcers, diarrhea, and other issues in the digestive tract.

ZES is considered rare, but it can occur at practically any stage of life, but primarily between the ages of 20 and 50.

The cause of ZES is unknown, but the sequence leading up to it is very predictable and clear. ZES is caused when tumors begin to grow in the pancreas, duodenum (the upper portion of the small intestines) or the lymph nodes adjacent to the pancreas.

The secretions that are produced by these organs are critical to effective digestion, but when they are created in too large quantities, excess acid is the result, which in turn leads to ZES. They can also lead to malignant tumors and other problems.

How is Zollinger-Ellison Syndrome Treated?

Clinicians perform an endoscopy and endoscopic ultrasound tests to locate ulcers and neoplasms. ImagingĀ  tests such as a CAT or MRI scan might be necessary to identify multiple gastrinomas.

A treatment plan is then designed to address the excessive production of gastric acids and heal ulcers with proton pump inhibitors (PPIs), a class of medications that is used to curb production of acids and which is also used to cure acid reflux or H2 blockers (usually not as efficient as PPIs).

Gastrinomas might require surgery to be removed, according to the number and location of the tumors. If malignant, a clinician might also opt for chemotherapy or radiotherapy.

The outlook for ZES varies greatly. 50% of the patients who had surgery are usually cured.

If gastrinomas are benign and grow slowly without affecting the liver (and spread to bones or skin) almost 85% of the patients survive for over fifteen years after being diagnosed.

The rate drops dramatically if the tumors are malignant and reach the liver.

Zollinger-Ellison Syndrome Prevention

Many scientists believe that ZES is an inherited predisposition. As a result, people who have blood relatives with the condition should be monitored for suspicious tumors. People with ZES are also often known to have tumors in their pancreas and other organs.

The exact cause of ZES is unknown. As a result, besides monitoring for the presence of suspicious tumors, there are few, if any, preventive measures that can be taken. Most doctors do recommend that patients who might be predisposed to ZES make sure that they have a healthy diet, exercise, and have regular medical check-ups.

Beyond this, those with ZES should monitor problems with diarrhea and other issues with the digestive tract. Anyone who suspected that they have ZES should consult with their doctor as soon as possible.

Last Reviewed:
September 10, 2016
Last Updated:
September 08, 2017