Abstract:Neuroendocrine tumors have a low prevalence and no specific symptoms, together with the lack of adequate research and understanding of them, leading to the unimproved survival of patients with this kind of disease over the past 30 years. In this paper, the authors overview the characteristics, diagnosis and management of pancreatic neuroendocrine tumors, based on the guideline of National Comprehensive Cancer Network (NCCN), and the consensus of European Neuroendocrine Tumor Society (ENETS) and North American Neuroendocrine Tumor Society (NANETS), in order to increase awareness of this condition.