Abstract
Intraductal papillary mucinous neoplasms (IPMN) are common and one of the main precursor lesions of pancreatic ductal adenocarcinoma (PDAC). PDAC derived from an IPMN is called intraductal papillary mucinous carcinoma (IPMC) and defines a subgroup of patients with ill-defined specificities. As compared to conventional PDAC, IPMCs have been associated to clinical particularities and favorable pathological features, as well as debated outcomes. However, IPMNs and IPMCs include distinct subtypes of precursor (gastric, pancreato-biliary, intestinal) and invasive (tubular, colloid) lesions, also associated to specific characteristics. Notably, consistent data have shown intestinal IPMNs and associated colloid carcinomas, defining the “intestinal pathway”, to be associated with less aggressive features. Genomic specificities have also been uncovered, such as mutations of the GNAS gene, and recent data provide more insights into the mechanisms involved in IPMCs carcinogenesis. This review synthetizes available data on clinical-pathological features and outcomes associated with IPMCs and their subtypes. We also describe known genomic hallmarks of these lesions and summarize the latest data about molecular processes involved in IPMNs initiation and progression to IPMCs. Finally, potential implications for clinical practice and future research strategies are discussed.
Highlights
Pancreatic ductal adenocarcinoma (PDAC) is one of the most lethal cancers, with a rising incidence in developed countries, and is projected to become the third and second leading cause of cancer-related deaths by 2025 in Europe and by 2030 in the United States, respectively [1,2]
When separately compared to conventional PDAC (cPDAC), colloid type intraductal papillary mucinous carcinoma (IPMC) were associated with significantly better outcomes, while no difference was observed between tubular type IPMC and cPDAC [16,30]
Both GNAS and KRAS mutations are found at similar rates across all grades of dysplasia [77], suggesting that they represent the very early events in the carcinogenesis of intraductal papillary mucinous neoplasms (IPMN) underlying the initiation of these neoplasms in most cases
Summary
Pancreatic ductal adenocarcinoma (PDAC) is one of the most lethal cancers, with a rising incidence in developed countries, and is projected to become the third and second leading cause of cancer-related deaths by 2025 in Europe and by 2030 in the United States, respectively [1,2]. IPMNs, that arise in the main pancreatic duct and/or their branches, are common lesions, with an estimated prevalence of 3–6% in the general population and more than 10% in older adults [6,7,8] These tumors represent the most common pancreatic cystic neoplasms undergoing resection [9,10]. Intraductal oncocytic papillary neoplasms, considered as a fourth subtype over the past decades, carry distinct genomic and morphological features. These are recognized a distinct entity apart from IPMNs since the 2019 WHO classification of tumors of the digestive system [12], and will not be discussed hereafter. We describe the molecular characteristics of IPMCs and discuss the more recent insights into the mechanisms of their onset and progression
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