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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. 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 and signs

  • Glucose intolerance (body has problem breaking down sugars)
  • High blood sugar (hyperglycemia)
  • 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

In most cases, the cancer has already spread to the liver when it is diagnosed.

Tests

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 level 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: Elsevier Saunders; 2010:chap 32.

National Cancer Institute: PDQ Pancreatic neuroendocrine tumors (islet cell tumors) treatment. Bethesda, MD: National Cancer Institute. Date last modified 11/10/2012. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/gastric/HealthProfessional. Accessed November 16, 2012.   


Review Date: 11/17/2012
Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, Stephanie Slon, and Nissi Wang.
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