Causes

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

Exams 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:

• CT scan of the abdomen
• Glucagon level in the blood
• Glucose level in the blood

Treatment

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

Outlook (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%.

Possible Complications

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

When to Contact a Medical Professional

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.

Updated: 4/28/2012

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.