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.
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 of glucagonoma may include any of the following:
- Glucose intolerance (body has problem breaking down sugars)
- High blood sugar (hyperglycemia)
- 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
Tests that may be done include:
Surgery to remove the tumor is usually recommended. The tumor does not usually respond to chemotherapy.
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%.
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.
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: www.cancer.gov/cancertopics/pdq/treatment/isletcell/HealthProfessional/page8. Accessed February 27, 2015.
Review Date: 11/26/2014
Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.