Development of Extrapancreatic Malignancy

Abstract

Over the past two decades, multiple studies have demonstrated an increased incidence of additional malignancies in patients with intraductal papillary mucinous neoplasm (IPMN). The majority of these additional cancers occur before or concurrent with the diagnosis of IPMN. The gastrointestinal tract is most commonly involved in secondary malignancies, with benign colon polyps and colon cancer commonly seen in Western countries and gastric cancer commonly seen in Asian countries. Other extrapancreatic malignancies associated with IPMN include benign and malignant esophageal neoplasms, gastrointestinal stromal tumors, carcinoid tumors, hepatobiliary cancers, breast cancers, prostate cancers, and lung cancers. There is no clear etiology for the development of secondary malignancies in patients with IPMN. Although population-based studies have shown different results from single-institution studies regarding the exact incidence of additional primary cancers in IPMN patients, both have reached the same conclusion: there is a higher incidence of extrapancreatic malignancies in patients with IPMN than in the general population. This finding has significant clinical implications for both the initial evaluation and subsequent long-term follow-up in patients with IPMN. At present, there are no recommended screening modalities for detecting extrapancreatic malignancies; however, once the diagnosis is made, the possibility of extrapancreatic neoplasms should be considered based on the frequency of malignancy in the general population of the country or region.