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       o May be crusty or scaly       o 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

     
  • Elevated serum glucagon level.  
  • Pancreatic tumor revealed by CT scan.  
  • Elevated fasting glucose level  
  • Abnormal glucose tolerance test

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

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.

Johns Hopkins patient information

Last revised: December 5, 2012
by Potos A. Aagen, M.D.

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