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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:
- 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 usually respond to chemotherapy.
Support Groups
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.
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