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Glucagonoma

Definition

Glucagonoma is a very rare tumor of the islet cells of the pancreas, which leads to an excess of the hormone glucagon in the blood.

Causes, incidence, and risk factors

Glucagonoma is usually cancerous (malignant). The cancer tends to spread and get worse.

This cancer affects the islet cells of the pancreas. As a result, the islet cells produce too much of the hormone glucagon.

The cause is unknown, but genetic factors play a role in some cases. A family history of the syndrome Multiple Endocrine Neoplasia type I (MEN I) is a risk factor.

Symptoms

  • Diarrhea
  • Excessive thirst (due to high blood sugar)
  • Frequent urination (due to high blood sugar)
  • Increased appetite
  • Inflamed mouth and tongue
  • Nighttime (nocturnal) urination
  • Skin rash on face, abdomen, buttocks, or feet that comes and goes, and moves around
    • May be crusty or scaly
    • May be raised sores (lesions) filled with clear fluid or pus
  • Unintentional weight loss

Signs and tests

Signs include:

Most of the time the cancer has already spread to the liver when it is diagnosed.

Tests may include:

Treatment

Surgery to remove the tumor is the preferred treatment. The tumor does not usually respond to chemotherapy.

Expectations (prognosis)

Approximately 60% of these tumors are cancerous. It is common for this cancer to spread to the liver. Only about 20% of people can be cured with surgery.

If the tumor is only in the pancreas and surgery to remove it is successful, patients have a 5-year survival rate of 85%.

Complications

The cancer can spread to the liver. High blood sugar levels can cause metabolic problems and tissue damage.

Calling your health care provider

Call your health care provider if you notice symptoms of glucagonoma.

References

Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier;2010:chap 32.

National Cancer Institute. Islet cell tumors (pancreatic) treatment PDQ. Updated October 31, 2008.


Review Date: 12/28/2010
Reviewed By: David C Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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