Glucagonoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10 year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis.

Natural History

• If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia.
• Glucagonoma have a very slow growth rate compared to most malignant tumors.

Prognosis

PROGNOSIS 

Glucagonomas are generally slow-growing, but are usually advanced by the time of diagnosis.

The most common site of metastasis is the liver, followed by regional lymph nodes, bone, adrenal gland, kidney, and lung.

Age, grade, and distant metastases are the most significant predictors of survival.

Once the tumor is metastatic, cure is rarely,

Five- and 10-year survival rates for patients undergoing resection of gastroenteropancreatic neuroendocrine tumors [2,12]

For approximately 60% of these tumors are cancerous, the prognosis of glucagonoma depends on the following:

• Whether or not the tumor can be removed by surgery
• The stage of the tumor, the size of the tumor, whether the cancer has spread outside the pancreas
• The patient’s general health
• Whether the tumor has just been diagnosed or has recurred
• The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10 year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis