Abstract
Intraductal papillary mucinous neoplasm (IPMN) was first recognized in the 1980s with increasing publications over the last decade as the incidence increased sharply, especially at tertiary-care referral centers. Population-based studies have estimated the age and sex-adjusted cumulative incidence of IPMN to be 2.04 per 100 000 person-years (95% confidence interval: 1.28-2.80). It is now understood that IPMN can be classified anywhere along the spectrum of the adenoma to carcinoma sequence and often harbors mutations in genes such as KRAS early in the disease process. Many patients are diagnosed incidentally after imaging of the abdomen for other diagnostic purposes. Patients that present with a history of symptoms such as pancreatitis and abdominal pain are at high risk of harboring a malignancy. Clinicopathologic features such as involvement of the main pancreatic duct, presence of mural nodules, and side branch disease > 3.0 cm in size may indicate that there is an underlying invasive component to the IPMN. In addition, the incidence of extra-pancreatic neoplasms is higher in patients with IPMN, with reported rates of 25% to 50%. There are no current screening recommendations to detect and diagnose IPMN but once the diagnosis is made, screening for extrapancreatic neoplasms such as colon polyps and colorectal cancer should be considered. Surgical resection is the recommend treatment for patients with high-risk features while close observation can be offered to patients without worrisome signs and symptoms of carcinoma.