glucagonoma

A glucagonoma usually forms in the tail of the pancreas.

In patients with glucagonoma, an endocrine tumor of the pancreas.

Fewer than 251 cases of glucagonoma have been described in the literature since their first description by Becker in 1942.

Glucagonoma is the third most common endocrine-secreting islet cell tumor, and 70% of glucagonomas are malignant.

Diabetes mellitus also frequently results from the insulin and glucagon imbalance that occurs in glucagonoma.

The only curative therapy for glucagonoma is surgical resection, where the tumor is removed.

Laparotomy is useful for obtaining histologic samples for analysis and confirmation of the glucagonoma.

When NME is identified in the absence of a glucagonoma, it may be considered "pseudoglucagonoma syndrome".

Diabetes mellitus is present in 80-90% of cases of glucagonoma, and is exacerbated by preexisting insulin resistance.

Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:

NME is considered part of the glucagonoma syndrome, which is associated with hyperglucagonemia, diabetes mellitus, and hypoaminoacidemia.

A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold overproduction of the hormone glucagon.

Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is the presenting problem in 70% of cases.

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It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.

Alpha cell tumors are commonly associated with glucagonoma syndrome, though similar symptoms are present in cases of pseudoglucagonoma syndrome in the absence of a glucagon-secreting tumor.

Abnormally elevated levels of glucagon may be caused by pancreatic tumors, such as glucagonoma, symptoms of which include necrolytic migratory erythema, reduced amino acids, and hyperglycemia.

It is associated with many conditions, including megaloblastic anemia from vitamin B deficiency, iron deficiency anemia (which in severe cases can lead to Plummer-Vinson syndrome), glucagonoma, and oral candidiasis.

Other associated endocrine involvements were pituitary adenomas in four (three with prolactin oversecretion), insulinomas in three, glucagonoma in one, and bilateral hyperplasia of the adrenal cortex in one.

The primary physiological effect of glucagonoma is an overproduction of the peptide hormone glucagon, which enhances blood glucose levels through the activation of anabolic and catabolic processes including gluconeogenesis and lipolysis respectively.

William Becker first described an association between NME and glucagonoma in 1942 and since then, NME has been described in as many as 70% of individuals with a glucagonoma.

Other examples of systemic disease capable of causing mouth ulcers include lupus erythematosus, Sweet syndrome, Reiter syndrome, Behçet syndrome, Wegener's granulomatosis, periarteritis nodosa, giant cell arteritis, diabetes, glucagonoma, sarcoidosis and periodic fever, aphthous stomatitis, pharyngitis and adenitis.