Abstract
Pancreatic neuroendocrine tumors are rare types of pancreatic cancer formed from islet cells of pancreas. Clinical presentation of pancreatic neuroendocrine tumors depends on both tumor progression and hormone secretion status, which generate several complications in both diagnosis and treatment. Despite numerous strategies, treatment of patients with pancreatic neuroendocrine tumors still needs improvement. It is suggested that immune response modulation may be essential in the regulation of pancreatic neuroendocrine tumor progression and patient's symptomology. Accumulating evidence indicates that immunotherapy seems to be a promising treatment option for patients with pancreatic neuroendocrine tumors. Nevertheless, several challenges in pre-clinical and clinical studies are present. This review provides knowledge about microenvironment of pancreatic neuroendocrine tumors including significance of cytokine and chemokine as well as specific immune cell types. Additionally, in vitro and in vivo models of pancreatic neuroendocrine tumors and translational challenges are highlighted.