Sporadically occurring functional pancreatic endocrine tumors: review of recent literature

Abstract

Pancreatic neuroendocrine tumors are rare neoplasms often associated with a clinical syndrome. Their rarity makes a comprehensive study difficult at any single institution, while their uniqueness makes them desirable for investigation. This review summarizes recent information and advancements concerning pancreatic neuroendocrine tumor diagnosis, imaging characteristics, treatment algorithms, and staging. Insulinomas and gastrinomas comprise the majority of functional pancreatic neuroendocrine tumors. Advances in their identification and diagnostic evaluation, imaging techniques, and treatment algorithms are presented. Furthermore, a new staging classification system has been proposed which may significantly improve the ability to conduct future multi-institutional investigations on pancreatic neuroendocrine tumors. Although rare, a thorough understanding of pancreatic neuroendocrine tumors is essential for all physicians due to the wide variety of symptoms with which patients present. Currently, patients are often misdiagnosed for extended periods of time. This review summarizes the recently published literature about diagnosis, imaging, treatment, and staging of pancreatic neuroendocrine tumors.