The diagnosis and management of intraductal papillary mucinous neoplasms of the pancreas: has progress been made?

Intraductal papillary mucinous neoplasms (IPMN) of the pancreas are premalignant mucin-producing epithelial tumors that arise from the pancreatic ductal system. These cystic tumors represent 15–30% of cystic lesions of the pancreas [Basturk et al. in Am J Surg Pathol 39(12):1730–1741, 1; Ferrone et al. in Arch Surg (Chicago, Ill: 1960) 144(5):448–454, 2, Kosmahl et al. in Virchows Arch Int J Pathol 445(2):168–178, 3; Spinelli et al. in Ann Surg. 239(5):651–657, 4]. It is believed that IPMN can progress from low-grade dysplasia to high-grade dysplasia to invasive cancer, and this pathway of progression accounts for 20–30% of pancreatic cancer [Adsay et al. in Am J Surg Pathol 28(7):839–848, 5; Tanaka et al. in J Gastroenterol 40(7):669–675, 6; Wu et al. in Sci Transl Med 3(92):92ra66, 7]. Furthermore, it is also widely believed that IPMN represent a field defect of the pancreas in which the entire ductal system is at risk of developing invasive carcinoma, not only in the area of radiographically detectable IPMN, and thus the remaining gland should undergo surveillance after partial pancreatectomy [Salvia et al. in Ann Surg 239(5):678–685, 8; Izawa et al. in Cancer 92(7):1807–1817, 9; Yamaguchi and Tanaka in Jpn J Clin Oncol 41(7):836–840, 10]. Increasingly, surgeons are faced with the dilemma between recommending highly complex resections—that have significant morbidity and mortality—in patients who may have low-risk IPMN (low-grade dysplasia), or alternatively, recommending observation for those who could possibly be harboring a radiographically occult malignancy. Given the complexity of the management decisions for patients with IPMN, the purpose of this paper is to review the current literature and to provide a summary of how accurate we are currently with the identification of high-grade dysplasia or progression to carcinoma in patients who present with IPMN.