• Zollinger-Ellison Syndrome

    (Z-E Syndrome; Gastrinoma)

    Definition

    Zollinger-Ellison syndrome is a rare disorder that arises from tumors and ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). These tumors, called gastrinomas, produce a large amount of gastrin. Gastrin is a hormone that causes the stomach to produce acid. With too much gastrin, excess acid is produced, causing ulcers in the stomach or small intestine.
    Gastric Ulcer
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    Gastrinomas occur as single tumors or small multiple tumors. Not only can these tumors lead to ulcers, they can also be cancerous (up to 66% malignant) and spread to the nearby lymph nodes or liver. This happens in about one-third to one-half of the cases of Zollinger-Ellison syndrome.
    About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). Patients with MEN 1 have additional endocrine tumors in the brain and neck.

    Causes

    The cause of Zollinger-Ellison syndrome is unclear. It is very rare; fewer than three out of a million people have the syndrome.

    Risk Factors

    Factors that may increase the chance of MEN 1 include:
    • Family history
    • Problems with the endocrine system
    • Recurrent peptic ulcer disease

    Symptoms

    Over 90% of people with Zollinger-Ellison syndrome have symptoms typical of a stomach ulcer.
    If you experience any of these symptoms, do not assume it is due to Zollinger-Ellison syndrome. These symptoms may be caused by other, less serious health conditions:
    • Abdominal pain
    • Nausea or vomiting
    • Diarrhea (30%)
    • Ulceration of stomach and small bowel
    • Heartburn, difficulty swallowing (60%)
    • Fatigue
    • Weight loss
    • Oily stool
    • GI bleeding with anemia, black stool, or bloody vomit

    Diagnosis

    Your doctor will ask about your symptoms and medical history, and perform a physical exam. He or she may also refer you to a gastroenterologist, a specialist who deals with gastrointestinal disorders.
    Tests may include the following:
      Gastrin stimulation tests
      • Standard test meal
      • Calcium
      • Secretin ( a hormone)
      Blood tests
      • Looking for elevated gastrin levels in blood
      Imaging Upper gastrointestinal endoscopy
      • A thin instrument with a light and camera is inserted down the throat and into the stomach and intestine to look for ulcers

    Treatment

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

    Surgical Removal of Tumor

    If there is only one tumor and it is not cancerous, a surgical removal may be attempted.

    Medications for Ulcers

    • Proton pump inhibitors are medications that reduce acid by blocking the ‘pumps’ in acid-secreting cells. Examples include Nexium, Prevacid, Protonix, and Prilosec.
    • Histamine blockers are medications to reduce the amount of hydrochloric acid released by the stomach. This will relieve pain and allow the ulcers to heal.
    • Chemotherapy such as streptozocin is used in those with malignant form. with or without evidence of cancer spread.
    • Control tumor growth with the use of somatostatin analogs (eg, octreotide).

    Prevention

    There are no know prevention steps since the cause is unclear.

    RESOURCES

    American Gastroenterological Association http://www.gastro.org/

    National Digestive Diseases Information Clearinghouse http://digestive.niddk.nih.gov/

    CANADIAN RESOURCES

    The Canadian Association of Gastroenterology http://www.cag-acg.org/

    Health Canada http://www.hc-sc.gc.ca/index%5Fe.html/

    References

    Berna MJ, Hoffmann KM, Long SH, et al. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National institutes of Health and comparison with 537 cases from the literature, evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine . 2006;85:331-64.

    Campana D, Piscitelli L, Mazzotta E, et al. Zollinger-Ellison syndrome: diagnosis and therapy. Minerva Med. 2005;96:167-206.

    DynaMed Editorial Team. Zollinger-Ellison syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated October 18, 2010. Accessed November 11, 2010.

    Mayo Clinic. Zollinger-Ellison syndrome. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/zollinger-ellison-syndrome/DS00461 . Updated July 24, 2010. Accessed November 11, 2010.

    Norton JA, Fraker DL, Alexander HR, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med . 1999;341:635-644.

    Norton JA, Jensen RT. Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison Syndrome. Ann Surg . 2004;240:757-773.

    Pellicano R, De Angelis C, Resegotti A, Rizzetto M. Zollinger-Ellison syndrome in 2006: concepts from a clinical point of view. Panminerva Med . 2006;48:33-40.

    Tomassetti P, Salomone T, Migliori M, et al. Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. Drgus Aging. 2003;20:1019-1034.

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