Zollinger-Ellison syndrome (patient information)

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Zollinger-Ellison syndrome

Overview

What are the symptoms?

Who is at highest risk?

Diagnosis

When to seek urgent medical care?

Treatment options

Where to find medical care for Zollinger-Ellison syndrome?

Prevention

What to expect (Outlook/Prognosis)?

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Jinhui Wu, M.D.

Overview

Zollinger-Ellison syndrome is also called gastrinoma. It is a rare disease characterized by one or more tumors in the pancreas, duodenum, or both. The tumors can cause the stomach to produce too much gastric acid, leading to peptic ulcers in the duodenum. Sometimes the tumors are cancerous and may spread to distant organs. Usual symptoms include burning abdominal pain, nausea and vomiting, weight loss and diarrhea. Treatmens include proton pump inhibitors and surgery.

What are the symptoms of Zollinger-Ellison syndrome?

Usual syptoms include:

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

Who is at highest risk?

The most potent risk factor in the development of Zollinger-Ellison syndrome is Multiple endocrine neoplasia type 1 (MEN1). Other risk factors include alcohol abuse.

Diagnosis

  • Gastric acid level and the amount of gastrin circulating in the blood
  • Upper gastrointestinal (GI) endoscopy: During this procedure, the doctor uses a thin, flexible, lighted tube with a small camera on the tip to see inside the upper GI tract.
  • Somatostatin receptor scintigraphy (SRS): In this test, tumor cells can light up after the radiotracer is injected into the bloodstream. The signal can be scaned by the gamma camera.
  • A computerized tomography (CT) scan: CT scans are often used to diagnose Zollinger-Ellison syndrome. It can confirm the location of the cancer, as well as distant organs where the cancer might have spread.
  • Findings on abdominal MRI suggestive of Zollinger-Ellison syndrome include solitary lesion or multiple lesions.
  • Ultrasonography can detect gastrinomas in 30%. Endoscopic ultrasonography (EUS) is a highly sensitive and specific procedure for the localization of pancreatic endocrine tumors and duodenal as well as pancreatic gastrinomas

Diseases with similar symptoms

When to seek urgent medical care?

Call your health care provider if symptoms of Zollinger-Ellison syndrome develop. If you experience either of the following symptoms, seeking urgent medical care as soon as possible:

Treatment options

The treatments include medications to relieve ulcer symptoms, surgery to remove tumors and chemotherapy. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities.

  • Proton pump inhibitors (PPI): This kind of drugs can effectively reduce gastric acid secretion in the stomach. Usual drugs include esomeprazole (Nexium), lansoprazole (Prevacid), pantoprazole (Protonix) and omeprazole (Prilosec).
  • Surgery: Surgical removal of gastrinomas is the only cure for Zollinger-Ellison syndrome.
  • Chemotherapy: For those the tumor can not be surgically removed, chemotherapy is available. The drugs include streptozotocin (Zanosar), 5-fluorouracil (Adrucil) and doxorubicin (Doxil).

Where to find medical care for Zollinger-Ellison syndrome?

Directions to Hospitals Treating Zollinger-Ellison syndrome

Prevention of Zollinger-Ellison syndrome

As a rare disease, the cause of Zollinger-Ellison syndrome is not clear. So the preventive measure is not known.

What to expect (Outlook/Prognosis)?

The outcome for patients with Zollinger-Ellison syndrome largely depends on the nature and extent of the disease.

References

http://www.nlm.nih.gov/medlineplus/ency/article/000325.htm

http://digestive.niddk.nih.gov/ddiseases/pubs/zollinger/

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