Glucagonoma natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2], Mohammed Abdelwahed M.D[3]

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. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.

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

Complications of glucagonoma include:

• Metastasis:
  • Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis.
  • Metastasis occurs mainly in the liver but in few cases, it can occur in lymph nodes, peritoneum, lung, and adrenals.
• Weight loss: due to the catabolic effect of glucagon, most patients lose weight.
• Anemia
• Neuropsychiatric manifestations include depression, dementia, psychosis, and agitation
• Reversible dilated cardiomyopathy^[1]

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 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.

Additionally:

• Age, grade, and distant metastases are the most significant predictors of survival.
• Five and 10-year survival rates for patients undergoing resection of gastroenteropancreatic neuroendocrine tumors.^[2]
• Sixty percent of glucagonomas are malignant. Once the tumor is metastatic, the cure is rare.^[3]

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