What is Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome is a rare disorder that usually occurs when a tumor called a gastrinoma develops in the pancreas or duodenum.

A gastrinoma secretes a hormone known as gastrin, which results in an overproduction of acid in the stomach.

Gastrinomas that result in Zollinger-Ellison syndrome may originate from the pancreas or, less likely, from the small intestine. They occasionally stem from other organs in the body, for example, the lymph nodes, stomach, liver, and ovaries.

According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), Zollinger-Ellison syndrome is rare. Approximately, one in every million people will develop the syndrome, but it is most common among men aged 30 to 50 years.

Someone who has Zolllinger-Ellison syndrome will likely develop severe, recurrent ulcers of the esophagus, stomach, and the duodenum and jejunum, which are the upper portions of the small intestine. The ulcers form because of the excess acid.

Symptoms

man with acid reflux
A person with Zollinger-Ellison syndrome develops tumors in the endocrine and pancreas that cause symptoms resembling those of a peptic ulcer.

Most people with Zollinger-Ellison syndrome have multiple tumors in the endocrine system, as well as tumors in the pancreas.

The signs and symptoms are similar to those of a peptic ulcer and include:

discomfort in the upper abdomen

burning and aching sensation in the upper abdomen

diarrhea

bleeding in the digestive tract

general weakness

black, “tarry” feces, resulting from bleeding in the digestive tract

nausea

unintentional weight loss

low appetite or feeling full too quickly

vomiting

Some people will experience heartburn, or gastroesophageal reflux, when gastric acid and food from the stomach backs up into the esophagus, which may be severe

High gastrin levels
A doctor will recommend tests to find out why the person has high gastrin levels and then recommend treatment.

People with chronic stomach inflammation and those who have recently undergone stomach surgery may have high levels of gastrin in the blood, even if the stomach does not produce much acid.

It is essential for the doctor to identify why the person has high gastrin levels, to find the correct treatment. They may test the acidity of the stomach.

If stomach acidity levels are not high, then it is highly unlikely the person has Zollinger-Ellison syndrome.

If the stomach is producing too much acid, a doctor may recommend a secretin stimulation test. This involves measuring gastrin levels, injecting the hormone secretin, and measuring the gastrin levels again.

If gastrin levels rise, this indicates that Zollinger-Ellison is present.

Upper gastrointestinal endoscopy

An upper gastrointestinal endoscopy involves a doctor inserting an endoscope into the person’s mouth, down the esophagus and into the stomach and duodenum to look for ulcers.

An endoscope is a long, thin tube that has light and video camera at the end that allows a doctor to see what is going on within particular parts of the body.

Other names for the procedure are:

upper endoscopy

upper GI endoscopy

esophagogastroduodenoscopy (EGD)

The doctor may also take a biopsy, or tissue sample, from the duodenum to test for gastrin-producing tumors.

The doctor will recommend fasting for a specific time before this procedure.

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