Epidemiology and genetics of pancreatic cancer

Pancreatic ductal adenocarcinoma (and its histological variants), also referred to as pancreatic cancer (PC) comprises 90% of exocrine pancreatic neoplasms [1]. This highly aggressive cancer is the fourth leading cause of cancer death in the USA [2]. More than 80% of patients with PC present with advanced disease that is incurable by surgery. Most tumors greater than 5 cm in size show disseminated metastases at presentation [3]. The 5-year survival rate of advanced PC is poor (< 5%) with a median survival of < 6 months. The 5-year survival rate improves to 20–30% in patients who harbor small, early invasive cancers (usually < 3 cm) and are candidates for surgical resection [2]. Thus, early diagnosis of PC before frank invasion occurs is critical to improve patient outcomes. Mucinous cystic neoplasms (MCNs) are mucin-secreting, cystic neoplasms of characteristic histopathology and variable clinico-biological profiles. They comprise 10–45% of cystic pancreatic neoplasms [4]. Intraductal papillary mucinous neoplasms (IPMNs) are characterized by cystic dilatation of ducts and intraductal papillary tumors with variable mucin production and tumor histobiology. Intraductal papillary mucinous neoplasms constitute 15–25% of cystic pancreatic neoplasms and typically show slow intra-luminal growth and low metastatic potential [4, 5]. Subsets of these two mucinous tumors progress to PC.