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Endocrine glands
Endocrine glands

Glucagonoma

Definition:

Glucagonoma is a tumor of the islet cells of the pancreas, which secrete the hormones insulin and glucagon.



Causes, incidence, and risk factors:

Glucagonoma is usually malignant, which means that it has a tendency to spread and get worse. The islet cells of the pancreas are affected by this cancer, and as a result, they produce too much of a hormone called glucagon.

The excess glucagon causes symptoms such as glucose intolerance and hyperglycemia (elevated blood sugar). Spreading (metastasis) to the liver may occur with this type of cancer. It also causes a distinctive skin lesion called necrolytic migratory erythema.

The cause is unknown, but genetic factors play a role in some cases. Risk factors include a family history of multiple endocrine neoplasia type I (MEN I).



Symptoms:
  • Skin rash that migrates on face, abdomen, perineum, buttocks, or lower extremities
    • May be crusty or scaly
    • May be raised lesions filled with clear fluid or pus
  • Inflamed mouth and tongue
  • Unintentional weight loss
  • Excess thirst
  • Frequent urination
  • Nocturnal (nighttime) urination
  • Increased appetite


Signs and tests:

Treatment:

Surgical removal of the tumor is the preferred treatment. The tumor does not respond to chemotherapy.



Support Groups:



Expectations (prognosis):

Approximately 60% of these tumors are malignant. Metastasis (spread) to the liver is common.



Complications:

Metastasis of cancer to the liver is a complication.



Calling your health care provider:

Call your health care provider if you notice symptoms suggestive of glucagonoma.



Prevention:

Awareness of risk, including family history, may allow early diagnosis.




Review Date: 8/3/2004
Reviewed By: Marcia S. Brose, M.D., Ph.D., Assistant Professor, Hematology/Oncology, The University of Pennsylvania Cancer Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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