Intraductal papillary neoplasm of the bile duct – A comprehensive review

Abstract Background Intraductal papillary neoplasm of the bile ducts is a rare tumor type. Management decisions are currently based upon a small case series. The authors have large own experience with IPNB. Objective The review aims at reporting on clinicopathological features of IPNB in order to provide guidance for management. Methods We searched PubMed, Medline, Microsoft Academic and Embase databases to identify studies of relevance. The analysis of own experience was also included. Results We analyzed 59 retrospective series and 25 cases from authors’ clinical experience. The main sign was jaundice and cholangitis, 33% and 48%, respectively. CT’s were performed in 63–76% and MR in 40–56%. Intraductal mass was found in 31–32% and duct dilatation in 27–30%. Endoscopic Retrograde Cholangio-Pancreatography (ERCP) was performed in 48–62%. IPNB with invasive carcinoma was found in 35.7–60% and IPNB with intraepithelial neoplasia in 36–60%. Histopathological confirmation before surgery was rare. The main treatment of IPNB is resection, in our material, both, hepatectomy and hepatectomy plus bile duct resections were performed in 40% of patients. The percentage of postoperative complications was 20%. The 5-year survival rate of all IPNB’s patients was 53.6%; in patients with associated invasive carcinoma - 22.2% and without invasive carcinoma - 100% (p ​= ​0.001). Conclusions Early surgery is advisable for radiologically suspected IPNB. The results of treatment depend on histopathology. They are worse at intraductal invasive carcinoma than at neoplasm with neoplasia.