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 peopleand was first identified in 1955 by Drs.
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Robert Zollinger and Edward Ellison in Columbus, Ohio.
As it enters the stomach, acid is present to help start to break down the food particles.
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.
With ZES, tumorscalled gastrinomasform in the duodenum and pancreas.
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.
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.
If needed, biopsies of any suspicious areas can be taken to be evaluated under the microscope.
This exam is often done under anesthesia.
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.
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 ofstomach acidbeing put into the stomach.
These medicationscalled proton pump inhibitorsinclude omeprazole,pantoprazole, and esomeprazole.
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?
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.
National Institute of Diabetes and Digestive and Kidney Diseases.Zollinger-Ellison syndrome.
1994;1(5):509-516. doi:10.1007/BF01211912
National Organization for Rare Disorders.Zollinger-Ellison syndrome.
