Background: Intraductal papillary mucinous adenocarcinoma (IPMCs) occur more frequently in main-duct intraductal papillary mucinous neoplasms. Methods: Review of the literature. Results: The prognosis of IPMCs depends on its histopathological subtype: colloid IPMCs have superior survival rates mainly secondary to more favorable pathological features, whereas tubular IPMCs have survival outcomes similar to that of conventional pancreatic adenocarcinomas. The epithelial background plays an equally important role in defining the biology of IPMCs: gastric IPMC subtypes demonstrate an overall worse survival outcome when compared to intestinal, pancreatobiliary, and oncocytic subtypes. Lymph node involvement is one of the strongest predictors of survival in IPMC, with a decreasing overall survival as the lymph node ratio increases. There is little evidence to support adjuvant chemoradiation in patients with IPMC. Conclusion: Our current understanding of IPMC biology based on histopathological and epithelial background subtypes as well as clinicopathological predictors should influence patient counseling and selection for adjuvant therapy.

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