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Glucagonoma

Definition

Glucagonoma is a very rare tumor of the islet cells of the pancreas, which release the hormones insulin and glucagon.

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, they produce too much of the hormone glucagon.

The cause is unknown, but genetic factors play a role in some cases. A family history of multiple endocrine neoplasia type I (MEN I) is a risk factor.

Symptoms

  • Diarrhea
  • Excess thirst
  • Frequent urination
  • Increased appetite
  • Inflamed mouth and tongue
  • Nighttime (nocturnal) urination
  • Skin rash on face, abdomen, buttocks, or lower extremities 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 respond to chemotherapy.

Expectations (prognosis)

Approximately 60% of these tumors are cancerous. Spread to the liver is common.

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.

Calling your health care provider

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

References

Simon P, Spilcke-Liss E, Wallaschofski H. Endocrine tumors of the pancreas. Endocrinol Metabl Clin North Am. 2006;35:431-437.

Review Date: 9/4/2008
Reviewed By: Sean O. Stitham, MD, private practice in Internal Medicine, Seattle, Washington; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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