Pathology diagnosis of pancreatic neuroendocrine tumors

Histopathology of pancreatic neuroendocrine tumors (PNETs) typically displays characteristic features. However, pathologists may encounter histological variants that may resemble other pancreatic tumors. Immunohistochemistry is a powerful tool in confirming neuroendocrine differentiation and differentiating PNETs with other pancreatic neoplasms. Histopathological features could be associated with inherited syndromes. Once the pathology diagnosis of neuroendocrine tumor was made, an accurate grading based on World Health Organization (WHO) classification is required. This review will focus on histology variants, immunohistochemistry and WHO classification of PNET.