Rare Functioning Pancreatic Endocrine Tumors

Abstract

The incidence of clinically detected PETs has been reported to be 4-12 per million, which is much lower than that reported from autopsy series (about 1%) [2, 3] .Considering functioning PETs, insulinomas are the most common (17% incidence), followed by gastrinoma (15%).The remainder incorporates RFTs and includes: VIPoma (2%), glucagonoma (1%), carcinoid (1%), somatostatinoma (1%), and the rest are comprised of adrenocorticotropic-secreting tumors (ACTHoma), GRFomas, calcitonin-producing tumors, parathyroid hormone-related peptide tumors, and other exceedingly rare neoplasms [4][5][6][7][8][9][10][11][12][13][14] .Similar to insulinomas and gastrinomas, the majority of RFTs are well-differentiated tumors [15] .Most RFTs present as malignant disease (WHO group 2) and liver metastases are common [8,10,14,16,17] .The 5-year survival rate is reported to be 60-100% for localized disease,