Glucagonoma
A
glucagonoma may be a rare
neuroendocrine tumor that originates almost exclusively within the pancreas and doubtless accounts for 1% of all
neuroendocrine tumors. Malignant glucagonomas are islet cell pancreatic
tumors that are discovered due to
glucagonoma syndrome (in which the
glucagonoma autonomously secretes glucagon), due to local mass effects, or incidentally.Glucagonomas originate from the alpha-2
cells of the pancreas. Unregulated production (overproduction) of
peptide hormones and growth factors, which are not normally expressed in the tissue of origin, is characteristic of
neuroendocrine tumors. Abnormal production of those bioactive
peptides can cause significant systemic toxic consequences and to the promotion of further
tumor growth. In 75-80% of cases, the
glucagonoma starts in malignant form, and in 50% of those cases, metastasis exists at diagnosis. In the majority of cases there's no known reason why a
glucagonoma occurs. However, during a small number of cases, the
glucagonoma is linked to an inherited condition called multiple endocrine neoplasia type 1 syndrome. Glucagonomas are extremely rare. In the UK, it's been estimated that every year one case of
glucagonoma is diagnosed per million people.If the
glucagonoma is confined to the pancreas then, in the majority of cases
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