Merck Manual

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Glucagonoma

By

Anthony Villano

, MD, Fox Chase Cancer Center

Reviewed/Revised Oct 2023
VIEW PROFESSIONAL VERSION

A glucagonoma is a tumor of the pancreas Pancreas The pancreas is an organ that contains two types of glandular tissue: Pancreatic acini Islets of Langerhans (See also Overview of the Digestive System.) The acini produce digestive enzymes.... read more Pancreas that produces the hormone glucagon, which raises the level of sugar (glucose) in the blood and causes a distinctive rash.

  • These tumors arise from cells in the pancreas that produce glucagon.

  • Symptoms are similar to those caused by diabetes, including weight loss and excessive urination.

  • Diagnosis includes blood and imaging tests.

  • Treatment is surgery and sometimes chemotherapy.

Glucagon is a hormone normally secreted by the pancreas when blood glucose levels fall. Glucagon simulates the liver to break down stored carbohydrates to increase blood glucose.

Symptoms of Glucagonoma

In addition, many people with glucagonoma have the very distinctive features of a chronic, brownish red rash called necrolytic migratory erythema and a smooth, shiny, bright red-orange tongue. The mouth also may have cracks at the corners. The rash, which causes scaling, starts in the groin and moves to the buttocks, forearms, hands, feet, and legs.

Diagnosis of Glucagonoma

Treatment of Glucagonoma

  • Surgical removal

  • Sometimes chemotherapy

  • Octreotide

Ideally, the tumor is surgically removed, which eliminates all symptoms. However, if removal is not possible or if the tumor has spread, chemotherapy may reduce the levels of glucagon and lessen the symptoms. However, chemotherapy does not improve survival.

The medication octreotide can be used to reduce glucagon levels, may clear up the rash, and may restore appetite, facilitating weight gain. But octreotide may elevate the levels of glucose in the blood even more.

Zinc taken by mouth, applied in an ointment, or given by vein (intravenously) may be used to treat the rash. Sometimes the rash is treated with intravenous amino acids or fatty acids.

Drugs Mentioned In This Article

Generic Name Select Brand Names
Bynfezia, Mycapssa, Sandostatin, Sandostatin LAR
NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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