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Zollinger-Ellison Syndrome

more about Zollinger-Ellison Syndrome


  • Zollinger-Ellison syndrome is a disease process associated with the development of ulcers due to over secretion of gastrin.  Gastrin -- a hormone that elicits acid secretion -- leads to the formation of ulcers by causing an excess of stomach acid.  Over two-thirds of these tumors are malignant and about one-third have usually spread to the liver by the time the disease is detected.  However, most of these tumors are potentially resectable (i.e., can be removed by surgery).

  • Over 90% of patients with this disease develop peptic ulcers.
  • The symptoms for Gastrinoma are identical to those for Peptic Ulcer Disease.
  • Abdominal pain (especially epigastric), Acid Reflux, fatty stool, right upper quadrant abdominal fullness (enlarged liver if there is metastasis) are common symptoms.
  • Usually the ulcers are single, but occasionally multiple ulcers develop. They can sometimes develop in unusual locations.
  • Some patients also develop Diarrhea and weight loss.
  • Patients should be tested for this disease if they do not respond to the usual ulcer treatments, have very large ulcers, have ulcers in unusual locations, have multiple ulcers, have recurrences of ulcers, or have high Calcium levels.  Also, patients with ulcers who are not taking anti-inflammatory drugs and are negative for bacterial infections of the stomach should be tested.
  • A quarter of patients with Gastrinomas develop multiple endocrine type neoplasia or tumors that produce various types of hormones.

  • Zollinger-Ellison syndrome is caused by tumors that oversecrete gastrin.  This causes an excess of acid to be produced, which results in ulcers.  The reason for the development of the tumors is not known.  However, some of the tumors are hereditary and others may be associated with other cancers.

  • Tests that might be done --
    1. Check the fasting gastrin level.
    2. Also check the acidity of the stomach.  If there is increased acid secretion along with high gastrin levels, then the likely diagnosis is Zollinger-Ellison syndrome.
    3. Once the diagnosis is made, the doctor should check the levels of other hormones that can sometimes be abnormal in patients with Zollinger-Ellison syndrome.
    4. To detect if the tumor has spread, the best test is somatostatin receptor scintigraphy or endoscopic Ultrasound (Ultrasound done from inside the stomach).  CT scan, MRI scan, and regular Ultrasounds are not as good at determining the location of the original tumor, nor are they very effective in identifying the spread of the tumor.

  • Metastatic disease --
    1. The most important factor predicting survival is whether the tumor has spread to the liver.
    2. If there are multiple areas of tumor in the liver, then controlling the oversecretion of acid is the number one concern.  This is done with strong anti-acid medications.
    3. If there is only one area of the liver with tumor, surgery is an option.  This will help decrease the need for anti-acid medicines.
    4. Even with spread to the liver, 30% of people live for 10 years.
  • Localized disease that has not spread to the  liver --
    1. Cure from the disease is possible only if the tumor is removed before it spreads to the liver.  Even if the tumor has spread to the lymph nodes, it does not affect survival.
    2. Cure is achieved by removing the tumor.  15-year survival for patients without spread to the liver is over 80 percent.

  • Carcinoid
  • VIPoma
  • Insulinoma
  • Glucagonoma
  • Somatostatinoma
  • Hypergastrinemia due to decreased acid secretion by the stomach or other causes must be ruled out.

more about Zollinger-Ellison Syndrome

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