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Glucagonoma

MEN I - glucagonoma

 

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

 

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

Tests that may be done include:

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

 

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.

 
  • Endocrine glands

    Endocrine glands - illustration

    Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

    Endocrine glands

    illustration

    • Endocrine glands

      Endocrine glands - illustration

      Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

      Endocrine glands

      illustration

    Tests for Glucagonoma

     

       

      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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