Neuroendocrine Tumors of the Pancreas

Neuroendocrine tumors of the pancreas are the second most common maLignant neoplasm of the pancreas. While they are less aggressive than ductal adenocarcinoma, they are often discovered at advanced cLinical stage, and overall survival is only 45% at 10 years. This tumor has recently received increased attention as pubLic figures such as Steve Jobs, cofounder of Apple Computer Corporation have succumbed to the disease. This article focuses on the cLinical and pathologic features of pancreatic neuroendocrine tumors, including diagnosis, classification, and treatment. In addition, genetic syndromes defined by or associated with the development of pancreatic neuroendocrine tumors are discussed. Finally, our current understanding of the molecular mechanisms underlying pancreatic neuroendocrine tumorigenesis is explored, and areas for future research are highLighted.