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Intraductal papillary mucinous neoplasms of the pancreas: a clinical challenge. INTRODUCTION: The incidental detection rate of intraductal papillary mucinous neoplasms (IPMNs) has significantly increased. However, little is known about the natural history of these tumors. Their optimal management and appropriate follow-up are still unclear. We have, therefore, reviewed the available literature on IPMN focusing on their diagnosis, treatment according to the risk of malignant transformation, and follow-up. Areas covered: Bibliographical searches were performed in PubMed for the terms 'intraductal papillary mucinous neoplasm' and 'natural history' and 'diagnosis' and 'treatment' and 'surgery' and 'follow-up' and 'prognosis.' PubMed was used to search for all the relevant articles published over the last 10 years. A total of 7244 records were identified. After filtering for year range, English language, human studies, article types, and removing duplicates, 74 articles were left with the strongest level of evidence. Expert commentary: Available guidelines for IPMN management are mainly based on expert opinions and may lack strong evidence. Further studies are warranted to better predict the risk of recurrence/future malignancy and to establish standardized guidelines. IPMNs management should be based on multidisciplinary discussion and treatment should be tailored to an individual patient according to patient and tumor characteristics.