Zollinger-Ellison Syndrome

What is Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome (ZES) causes severe peptic ulcer disease and chronic diarrhea. It affects people who have gastrinomas. These are a type of neuroendocrine tumors in your pancreas and duodenum (the top part of your small intestine).

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. About 60% of gastrinomas are cancerous. Your healthcare provider may call ZES pancreatic ulcerogenic tumor syndrome.

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

What are the symptoms of Zollinger-Ellison syndrome?

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

• Blood in your vomit or poop from gastrointestinal bleeding
• Burning or gnawing stomach pain from acid reflux
• Fatigue and weakness from anemia that gastrointestinal bleeding may cause
• Greasy, fatty poops
• Heartburn
• Indigestion, especially after you eat fatty foods
• Loss of appetite
• Nausea and vomiting
• Unexplained weight loss

Your symptoms depend on the tumor size and how many tumors you have. Large gastrinoma tumors release more gastrin, so your stomach produces 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 (pruritus) and yellowish skin from jaundice. Jaundice can also make the whites of your eyes (sclera) and mucous membranes turn yellow.

What causes Zollinger-Ellison syndrome?

Most cases of Zollinger-Ellison syndrome happen sporadically (for no known reason). About 25% of people with this condition have MEN Type 1. MEN stands for multiple endocrine neoplasia. MEN is a rare inherited disorder that 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 do healthcare providers diagnose Zollinger-Ellison syndrome?

A healthcare provider will do a physical exam. They’ll ask about your symptoms and your health history. They may order the following tests:

• Blood tests. A blood test may detect high gastrin levels in your blood (hypergastrinemia).
• Imaging tests. A provider may order CT scans or MRI scans 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 preliminary tests detect tumors, your provider may order more tests, including:

• Endoscopy. They may order an upper endoscopy to examine 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. They may do this to get a sample of tumor tissue for a medical pathologist to examine.

What are the treatment options for Zollinger-Ellison syndrome?

Your treatment will depend on your symptoms and 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 Zoller-Ellison syndrome. Proton pump inhibitors reduce the amount of gastric acid your stomach produces.

Surgery

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

• Duodenectomy. Your surgeon makes a small incision in your duodenum to remove the tumor.
• Gastrectomy. This surgery removes all or part of your stomach.
• Liver surgery. Your surgeon removes tumors in your liver.
• Pancreatectomy. This surgery removes all or part of your pancreas.
• Tumor enucleation. You may have this surgery if there are tumors in the head of your pancreas.

Cancer therapy

Your provider may recommend chemotherapy. This treatment may ease symptoms and slow down how quickly the gastrinomas spread.

Can treatment cure Zoller-Ellison syndrome?

Surgery to remove cancerous gastrinomas may cure the condition. But a cure depends on removing all 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?

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 90% of people will live between five and 10 years after surgery removes all gastrinoma tumors. The five-year survival rate estimate drops to 43% in cases where 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.

How do I take care of myself?

That depends on your situation. Most people will always need medication to reduce gastric acid. Zollinger-Ellison syndrome happens because you have gastrinoma tumors. Most of these tumors are cancerous. If you have cancerous tumors, you’ll need follow-up care to confirm the tumors haven’t come back.

When should I see my healthcare provider?

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