RT Book, Section A1 Dillhoff, Mary E. A1 Ellison, E. Christopher A2 Zinner, Michael J. A2 Ashley, Stanley W. A2 Hines, O. Joe SR Print(0) ID 1160046014 T1 Endocrine Tumors of the Pancreas T2 Maingot's Abdominal Operations, 13e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9780071843072 LK accesssurgery.mhmedical.com/content.aspx?aid=1160046014 RD 2024/10/05 AB Pancreatic neuroendocrine tumors (PNETs), traditionally termed islet cell tumors, are rare cancers occurring in approximately 1000 patients per year in the United States, representing 3% of all pancreatic tumors.1 The incidence of neuroendocrine tumors has increased over the last three decades, likely from increased use of and improvement in imaging modalities.2-4 The incidence overall of PNETs is increasing from .17 per 100,000 people in 1973 to .47 per 100,000 people in 2007.5 The peak incidence for PNET is between the ages of 40 and 69 years. While survival is significantly longer than patients with pancreatic adenocarcinoma, once patients have metastatic disease cure is not likely. However, surgical treatment plays a very important role in palliation of symptoms from hormone-producing tumors. Overall survival rate of resected tumors is 55%, but is only 15% with metastatic disease. Surgical resection for localized disease is the only curative treatment. The majority of PNETs are nonfunctional; however, some may secrete active gastrointestinal hormones that produce clinical syndromes. Most tumors are sporadic; however, some are associated with syndromes such as multiple endocrine neoplasia (MEN), von Hippel–Lindau syndrome, neurofibromatosis, tuberous sclerosis, and von Recklinghausen syndrome. Tumors associated with MEN syndromes are more likely to be aggressive and multifocal.