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.
MEN I - glucagonoma
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
The health care provider will perform a physical exam and ask about your medical history and symptoms.
Tests that may be done include:
Surgery to remove the tumor is usually recommended. The tumor does not usually respond to chemotherapy.
You can ease the stress of illness by joining a cancer support group. Sharing with others who have common experiences and problems can help you not feel alone.
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 provider if you notice symptoms of glucagonoma.
National Cancer Institute website. Pancreatic neuroendocrine tumors (islet cell tumors) treatment (PDQ) - health professional version. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq. Updated February 8, 2018. Accessed November 12, 2018.
Schneider DF, Mazeh H, Lubner SJ, Jaume JC, Chen H. Cancer of the endocrine system. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff's Clinical Oncology. 5th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 71.
Vella A. Gastrointestinal hormones and gut endocrine tumors. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 13th ed. Philadelphia, PA: Elsevier; 2016:chap 38.
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.