Intraductal papillary mucinous neoplasm of the liver: GI image.

Intraductal papillary mucinous neoplasms (IPMNs) are rare, mucin-producing, predominantly noninvasive tumors arising from epithelial cells. Most IPMNs arise from the pancreas. There exists a subset of IPMN of the biliary tract (BT-IPMN). IPMNs regardless of origin produce large amounts of mucin relative to scant amounts of epithelial cells, leading to mass effect disturbances in bile flow. Affected bile ducts exhibit marked dilatation. The majority of IPMN patients present with a non-malignant neoplasm. Because of potential for transformation, surgery is the treatment of choice. Bronchobiliary fistulas can present as congenital defects, following thoracoabodominal trauma, or as rare complications of diseases of the biliary tract such as BT-IPMN. There are no reported cases in the literature of bronchobiliary fistula associated with BT-IPMN, but there is a clear theoretical risk.