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What is a glucagonoma?

About 5-10% of pancreatic neuroendocrine tumors are glucagonomas, tumors that produce an inappropriate abundance of the hormone glucagon. It is estimated that one person out of 20 million will develop a glucagonoma each year. Glucagon is a protein segment that is produced by the alpha cells of the islets of Langerhans, the endocrine elements of the pancreas. The role of glucagon is to balance the effects of insulin, keeping blood sugar levels normal. Patients with glucagonomas are classically 40-70 years of age, with no significant preference for gender. Most of these tumors develop spontaneously in patients with no family history of endocrine tumors, although a few may occur in the setting of the MEN1 syndrome. These tumors originate in the pancreas, usually in the body or tail of the gland.

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