Citation, DOI and article data
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.
Treatment and prognosis
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.
- 1. Demos TC, Posniak HV, Harmath C et-al. Cystic lesions of the pancreas. AJR Am J Roentgenol. 2002;179 (6): 1375-88. AJR Am J Roentgenol (full text) - Pubmed citation 2. Makar AB, McMartin KE, Palese M, Tephly TR. Formate assay in body fluids: application in methanol poisoning. (1975) Biochemical medicine. 13 (2): 117-26. doi:10.1016/0006-2944(75)90147-7 - Pubmed 3.Lv WF, Han JK, Liu X, Wang SC, Pan BO, Xu AO. Imaging features of glucagonoma syndrome: A case report and review of the literature. (2015) Oncology letters. 9 (4): 1579-1582. doi:10.3892/ol.2015.2930 - Pubmed