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Glucagonoma - All Information

Definition of Glucagonoma:

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:

Glucose intolerance
High blood sugar (hyperglycemia)

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.

Endocrine glands

Reviewed last on: 9/4/2008
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.

References

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

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