Last revised by Jeremy Jones on 20 Sep 2021

Glucagonomas are pancreatic endocrine tumors that secrete glucagon. Most lesions are malignant.

They are very rare with an incidence of ~0.000005% or less than 1 case per 20 million. Equal incidence in middle-aged men and women. 

Accounts for 1% of all the neuroendocrine tumors and less than 5% of all primary pancreatic malignancies 2.

Most patients present with a necrolytic migratory rash and various other elements of the 4D syndrome which includes:

Stomatitis, diarrhea, anemia, and weight loss may also occur.

Plasma level of glucagon is elevated (>500 pg/mL).

Tumor size is variable, but most are large (>5 cm) and have metastasized at the time of diagnosis. Most are located in the distal pancreas and tend to demostrate significant hypervascularity. 

Tumors may be solid or contain central low-attenuation areas on CT.

The MR features of these lesions have not yet been reported. 

Standard treatment is surgical resection, depending on its location the approach can be divided into local resection, pancreactic body and tail resection and pancreatoduodenectomy. 

Approximately 50% of patients survive for at least 5 years post-diagnosis.

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