Intraductal papillary neoplasms of the bile duct: A European retrospective multicenter observational study (EUR-IPNB Study).

[en] BACKGROUND/PURPOSE: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare disease in Western countries. The main aim of this study was to characterize current surgical strategies and outcomes in the mainly European participating centers.
METHODS: A multi-institutional retrospective series of patients with a diagnosis of IPNB undergoing surgery between January 1, 2010, and December 31, 2020 was gathered under the auspices of the E-AHPBA. Textbook outcome was defined as non-prolonged length of hospital stay plus absence of any Clavien-Dindo grade ≥III complication, readmission, or mortality within 90 postoperative days.
RESULTS: A total of 28 centers contributed 85 patients who underwent surgery for IPNB. Median age was 66 years (55-72), 49.4% were women and 87.1% Caucasian. Open surgery was performed in 72 patients (84.7%), laparoscopic in 13 (15.3%). Textbook outcome was achieved in 54.1% of patients, reaching 63.8% after liver resection, and 32.0% after pancreas resection. Median overall survival was 5.72 years, with 5-year overall survival of 63% (95% CI 50-82). Overall survival was better in patients with Charlson comorbidity score ≤4 versus >4 (P=0.016), intra- versus extra-hepatic tumor (P=0.027), single versus multiple tumor (P=0.007), those who underwent hepatic versus pancreatic resection (P=0.017), or achieved versus failed textbook outcome (P=0.029). Multivariable Cox regression analysis showed that not achieving textbook outcome (HR 4.20, 95% CI 1.11-15.94, P=0.03) was an independent prognostic factor of poor overall survival.
CONCLUSIONS: Patients undergoing liver resection for IPNB were more likely to achieve a textbook outcome than those requiring a pancreatic resection. Comorbidity, tumor location and tumor multiplicity influenced overall survival. Textbook outcome was an independent prognostic factor of overall survival.