Intraductal papillary mucinous tumors of the pancreas

Abstract

Intraductal papillary mucinous neoplasms of the pancreas (IPMNs) are increasingly recognized in clinical practice. They are a unique clinicopathologic entity that is characterized by mucin production, cystic dilation of the pancreatic ducts, and intraductal papillary growth. Histologically, they may demonstrate a spectrum of cellular atypia ranging from minimal mucinous hyperplasia to frank invasive carcinoma. Similar to invasive ductal adenocarcinoma of the pancreas, patients with IPMN generally present in the seventh or eighth decade of life with the head of the gland being the predominant disease site. The majority of patients are symptomatic at presentation with abdominal pain, weight loss, and diarrhea being the predominant symptoms. Traditionally, the diagnosis was made following an endoscopic retrograde cholangiopancreatography. However, a cross-sectional imaging study such as a computed tomography or magnetic resonance imaging is now the radiological imaging studies of choice. Current thinking suggests that at best IPMN represents a premalignant condition and, as such, surgical intervention should be recommended. The aim should be to resect all gross disease while attempting to achieve a negative surgical margin, which in the majority of cases can be achieved by a partial pancreatectomy. The disease seems to be somewhat indolent with resection of noninvasive disease being curative in the majority of patients with 5- and 10-year actuarial survival rates of 75% and 60% reported. However, up to 50% of cases will have invasive cancer, and for this cohort the prognosis is more guarded. The role of adjuvant therapies is unclear, and further studies are required in this area.