Zollinger-Ellison Syndrome (Gastrinoma)

Zollinger-Ellison syndrome is a rare digestive disorder caused by a tumor of the pancreas called gastrinoma.

Symptoms of Gastrinoma

The symptoms of Zollinger-Ellison syndrome are:

  • Chronic diarrhea
    Recurrent diarrhea is the only symptom of this condition in about 30% of patients. This symptom may also precede the development of Zollinger-Ellison syndrome by several years.

  • Pain in the stomach and esophagus, especially at night or between meals.
    This pain may go away or worsen with eating.

  • Heartburn or pain in the chest and upper abdomen
  • Nausea
  • Vomiting of blood, mucus, coffee-ground like vomit (digested blood)
  • Difficulty in swallowing
  • Sore throat, coughing, and wheezing
  • Sour or bitter taste in the mouth
  • Blood in the stool
  • Black, tar-like or deep-red colored stool
  • Steatorrhea, or froty, oily stool with particularly bad odor (evidence of malabsorption of nutrients)
  • Malnourishment

How Is It Diagnosed?

Your doctor would perform the following tests to diagnose Zollinger-Ellison syndrome:

  • Endoscopy
    A flexible tube with camera mounted at the end, called an endoscope, is inserted through the esophagus, stomach, and small intestine. Persistent and chronic ulcer, or an ulcer at a rare location such as the end of the small intestine may be signs of this condition.

  • Stomach acid test
    Elevated levels of acid in the stomach is a sign of this condition.

  • Blood test
    Elevated levels of the hormone gastrin in the blood is a sign of Zollinger-Ellison syndrome.

In a test called secretin stimulation test, an injection of the hormone secretin is performed before the blood test to check the level of gastrin.

The location of the tumor is identified by the following tests:

  • Computed Tomography or CT Scan
  • Magnetic Resonance Imaging or MRI

Causes of Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome is caused by a tumor (or tumors) called gastrinoma that affects commonly the pancreas. In rare cases, this tumor is also found in the stomach, liver, small intestine, and ovaries. The tumor produces a high amount of the hormone gastrin, which cause over-production of stomach acid.

It is though that there is a strong genetic component to this condition. People with a history of chronic peptic ulcer or cancer of the pituitary glands and parathyroid are at increased risk of developing this disease.

Zollinger-Ellison syndrome may also be a part of a larger condition called multiple endocrine neoplasia-I, an inherited form of cancer with tumors not only in the digestive tract, but also the pituitary glands and parathyroid.

Treatment for Zollinger-Ellison syndrome?

The treatments for this condition include:

  • Medications
    Prescription medicines, such as proton pump inhibitors that stop the production of stomach acid can treat the symptoms of Zollinger-Ellison syndrome.

  • Chemotherapy

  • Surgery
    Elimination of the tumor may require the removal of part of the stomach, intestines, pancreas, and liver. In very rare cases, total stomach removal or gastrectomy may be necessary, although in most cases, prescription proton pump inhibitors are sufficient.

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