Pancreatic neuroendocrine tumors (PanNETs) are the second most common malignant tumor of the pancreas. Neuroendocrine tumors are neoplasms that arise from cells of the endocrine and nervous systems. They all share common features such as having special secretory granules and producing biogenic amines and polypeptide hormones. The mean age of PanNETs occurrence is of 58 years old, and although their prognosis are better than that of pancreatic ductal adenocarcinoma, they are still poor tumors with an average overall 5 years survival only 42%. PanNETs are well classified into functional versus non-functional tumors. While functional PanNETsare easily to be recognized by their classic clinical presentations such as Whipple’s triad for insulinoma or the migratory necrolytic erythema for glucagonoma, patients with non-functional PanNETs usually do not suffer from symptoms due to hormone hypersecretion, even if hormone levels are elevated on laboratory evaluation. Although several TNM classification systems for PanNETs patients have been established, a recent study suggests that the ENETs (European Neuroendocrine Tumor Society) TNM classification was superior to the AJCC (American Joint Committee on Cancer) or the WHO2010 grading system. This review aims to present those classification systems as well as list important genetic signatures in PanNETs.
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