Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver. About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.

  • Causes


    Zollinger-Ellison syndrome is caused by gastrin-producing tumors, called gastrinomas. Gastrin is a hormone that stimulates the stomach to produce acid. Excess acid production may lead to ulcers
    in the
    stomach
    or
    duodenum.

  • Definition

    Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver.

    About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.

    Gastric Ulcer
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  • Diagnosis

    Your doctor will ask about your symptoms and medical history. A physical exam will be done.

    Tests may include:

    • Blood tests—specifically to look for elevated levels of the hormone gastrin or evidence of MEN 1
    • Upper gastrointestinal endoscopy—a flexible tube with a light and camera is inserted down the throat and into the stomach and intestine to look for ulcers

    Imaging

    tests may help your doctor to detect and localize the tumors. These tests may include:

    • MRI
    • Abdominal CT scan
    • Abdominal ultrasound
    • Somatostatin receptor scintigraphy
    • Angiography

  • Prevention

    There are no current guidelines to prevent Zollinger-Ellison syndrome. If you have a family history of Zollinger-Ellison syndrome or MEN 1, consider contacting a genetic counselor for screening.

  • Risk Factors

    Factors that increase your chance of getting Zollinger-Ellison syndrome include:

    • Your or family members with MEN 1
    • History of endocrine disorders
    • Recurrent peptic ulcers

  • Symptoms


    In many people, Zollinger-Ellison syndrome causes symptoms similar to an ulcer or
    gastroesophageal reflux disease
    (GERD).

    Symptoms may include:

    • Abdominal pain
    • Heartburn
    • Nausea or vomiting
    • Fatigue
    • Diarrhea
    • Weight loss
    • Blood in the vomit or stool

  • Treatment

    Talk with your doctor about the best treatment plan for you. Options include: