Intraductal papillary neoplasm of the bile duct - A comprehensive review.

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.