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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.

Alternative Names

MEN I - glucagonoma

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

The health care provider will perform a physical exam and ask about your medical history and symptoms.

Tests that may be done include:

Treatment

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

Support Groups

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.

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 provider if you notice symptoms of glucagonoma.

References

National Cancer Institute. Pancreatic neuroendocrine tumors (islet cell tumors) treatment (PDQ) - health professional version. Updated April 30, 2015. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq#section/all. Accessed December 21, 2016.

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.

Review Date:11/10/2016
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.

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Outcome Data

No data available for this condition/procedure.

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