• 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
    • 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
    • Symptoms of glucagonoma may include any of the following:

      • 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
      • Weight loss

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

  • Exams and Tests
  • Treatment
    • Surgery to remove the tumor is usually recommended. 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, people have a 5-year survival rate of 85%.

  • Possible Complications
    • The cancer can spread to the liver. High blood sugar level can cause problems with metabolism 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: Elsevier Saunders; 2010:chap 32.

      National Cancer Institute: PDQ Pancreatic neuroendocrine tumors (islet cell tumors) treatment. Bethesda, MD: National Cancer Institute. Date last modified March 7, 2014. Available at: Accessed February 27, 2015.