A Unique Presentation of Diffuse Intraductal Papillary Mucinous Neoplasm of Bile Duct Successfully Treated With Orthotopic Liver Transplant and Pancreaticoduodenectomy Procedure: A Case Report.

Biliary intraepithelial neoplasm (BilIN) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are recognized as the two main precursors to biliary carcinoma. Surgical resection is the mainstay of treatment, with pancreaticoduodenectomy for extrahepatic biliary duct disease involving the pancreatic or distal portion of the bile duct, and bile duct and liver resection for perihilar and intrahepatic bile duct involvement. For diffuse IPMN-B with involvement of the entirety of the biliary epithelium of both intrahepatic and extrahepatic biliary systems, there is no well-documented consensus on treatment due to its rarity. Therefore, we present a case of a 56-year-old male with diffuse IPMN-B managed with combined orthotopic liver transplant and pancreaticoduodenectomy. As such, the clinical presentation, diagnostics, and unique course of intervention for our patient are described. This case provides insight into the topic of managing IPMN-Bs, particularly with diffuse biliary tree involvement. With such a rare disease with wildly varied presentations, consensus on a set treatment algorithm is nearly impossible to establish. This case describes one treatment pathway we found to be successful.