Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome (ZES) is a condition that occurs when a tumor known as a gastrinoma releases too much of a hormone called gastrin. Another name for ZES is pancreatic ulcerogenic tumor syndrome.

Gastrinomas are a type of neuroendocrine tumor. They’re mainly in your pancreas and duodenum (the top part of your small intestine). About 6 out of every 10 gastrinomas are cancerous.

Gastrinoma tumors affect the cells that release gastrin. This hormone makes your stomach release gastric acid. Too much gastric acid in your stomach causes ulcers, acid reflux and other issues. It can lead to severe peptic ulcer disease and chronic diarrhea.

Treatment can ease Zollinger-Ellison symptoms. Surgery to remove gastrinoma tumors can cure it. But the tumors that cause them can come back.

Zollinger-Ellison syndrome symptoms

Zollinger-Ellison syndrome may not cause symptoms. When it does, symptoms may include:

• Blood in your vomit or poop
• Burning or gnawing stomach pain
• Fatigue and weakness
• Greasy, fatty poops
• Heartburn
• Indigestion, especially after you eat fatty foods
• Loss of appetite
• Nausea and vomiting
• Unexplained weight loss

The symptoms you experience will depend on how many tumors you have and how big they are. Large gastrinoma tumors release more gastrin. So, your stomach will produce more gastric acid. Large amounts of gastric acid in your stomach can lead to more severe peptic ulcer disease symptoms.

Tumors that spread (metastasize) to your liver may cause liver cancer. Liver cancer symptoms include:

• Itchy skin
• Yellowish skin from jaundice
• Yellow sclera (the whites of your eyes) and mucous membranes

What is the cause of Zollinger-Ellison syndrome?

Most cases of Zollinger-Ellison syndrome happen randomly (for no known reason). But about 1 in every 4 people with this condition has a rare genetic disorder called multiple endocrine neoplasia (MEN) type 1. MEN affects glands in your endocrine system.

What are the complications of Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome can be life-threatening. For example:

• A gastrinoma tumor that blocks your duodenum can make it rupture
• The tumor can spread to your liver
• Large amounts of gastric acid may lead to severe gastrointestinal bleeding

How to diagnose Zollinger-Ellison syndrome

A healthcare provider will do a physical exam and ask you about your symptoms and health history. They may order certain tests, including:

• Blood tests: A blood test may detect high gastrin levels in your blood (hypergastrinemia).
• Imaging tests: A provider may order CT scans or MRIs to look for gastrinoma tumors in your pancreas or duodenum.
• Stomach acid test: In this test, a provider inserts a tube through your nose or throat into your stomach to get gastric acid samples.

If these tests find tumors, your provider may order more tests, including:

• Endoscopy: In an upper endoscopy, your provider examines your upper GI tract, pancreas and duodenum.
• Endoscopic ultrasound: This test combines ultrasound and endoscopy to provide more detailed images.
• ERCP: Providers use this imaging test to look inside your bile ducts and pancreatic ducts.
• Fine-needle aspiration: Your provider may do this test to get a sample of tumor tissue for a medical pathologist to examine.

How is Zollinger-Ellison syndrome treated?

Treatment for Zollinger-Ellison syndrome depends on the symptoms you have. It also varies based on whether the gastrinomas are cancerous or noncancerous. Treatment options include medication, surgery and cancer therapy.

Medication

Proton pump inhibitors (PPIs) are the most common medication for Zollinger-Ellison syndrome. PPIs reduce the amount of gastric acid your stomach makes.

Surgery

Your provider may recommend surgery to remove the tumor. Surgeries to remove gastrinoma tumors include:

• Duodenectomy: Your surgeon will make a small cut (incision) in your duodenum to remove the tumor.
• Gastrectomy: This is surgery to remove all or part of your stomach.
• Liver surgery: Your surgeon will remove tumors in your liver.
• Pancreatectomy: This is surgery to remove all or part of your pancreas.
• Tumor enucleation: You may need this surgery if there are tumors in the head of your pancreas.

Cancer therapy

Your provider may recommend chemotherapy. This treatment may ease symptoms. It can also slow down the gastrinomas’ spread.

Can Zollinger-Ellison syndrome be cured?

Surgery to remove cancerous gastrinomas may cure the condition. But a cure depends on removing all the cancerous cells in your body. If surgery is successful, you’ll need to take medication to manage gastric acid for the rest of your life.

What can I expect if I have Zollinger-Ellison syndrome?

You should contact your provider if Zollinger-Ellison syndrome symptoms get worse after treatment or come back.

Your prognosis, or what you can expect after treatment, depends on the tumor type. Noncancerous gastrinoma tumors aren’t life-threatening. You may need ongoing treatment to manage stomach acid levels.

The situation changes if you have cancerous tumors. In that case, you may need surgery and follow-up cancer treatment.

What are survival rates for Zollinger-Ellison syndrome?

Zollinger-Ellison survival rates vary. Experts estimate more than 9 out of every 10 people will live between five and 10 years after surgery removes all gastrinoma tumors. The five-year survival rate estimate drops to about 4 in every 10 cases when surgery doesn’t remove all tumors. It’s important to remember that cancer survival rates are estimates. If you have ZES, ask your healthcare provider what you can expect.