Clinicopathologic characteristics and long-term prognosis of intraductal papillary neoplasm of the bile duct: a retrospective study

BACKGROUND: Intraductal papillary neoplasm of the bile duct (IPNB) is a premalignant neoplasm that can involve both the intrahepatic and extrahepatic bile ducts. Owing to the low incidence and confusing nomenclature, its clinicopathological features remain controversial. Additionally, only a few studies have reported on the long-term prognosis of IPNB to date. Therefore, the present study aimed to clarify the clinicopathological characteristics and prognosis of IPNB. METHODS: Medical records of patients with IPNB treated at our hospital between August 2000 and October 2021 were retrospectively reviewed. A database of demographic characteristics, test results, surgical details, pathological findings, and follow-up information was constructed for analysis. Patients were divided into intrahepatic and extrahepatic groups, and dysplasia and invasive carcinoma groups for comparison. Differences between study groups were analyzed using the χ(2) test, Fisher’s exact test, t-test, or Mann–Whitney U test, as appropriate. Cumulative survival rates were estimated using the Kaplan–Meier method. RESULTS: In total, 43 patients (21 men and 22 women) with IPNB were included in the study. The median age at diagnosis was 62 (54–69) years. Thirty-eight patients underwent surgery. The mean operation time was (269.5 ± 94.9) min. Five patients underwent endoscopic retrograde cholangiopancreatography for biopsy. Twenty-one and 22 patients had intrahepatic and extrahepatic lesions, respectively. The extrahepatic group had more patients with intraluminal masses (p = 0.021) and abnormal bilirubin levels (p = 0.001), but fewer patients with hepatolithiasis (p = 0.021). The operation time was longer in patients with extrahepatic lesions (p = 0.002). Twenty patients had dysplasia and 23 had invasive carcinoma. The invasive carcinoma group had a longer operation time than the dysplasia group (p = 0.004). As of March 2022, 39 patients were followed up, with a mean follow-up time of (56.2 ± 38.2) months. Fifteen patients survived without tumors, two survived with tumors, and 22 patients died. The 1-, 3-, 5-, and 10-year cumulative overall survival rates were 86.9%, 65.8%, 49.8%, and 32.0%, respectively. CONCLUSIONS: IPNB is a rare bile duct disease that occurs mainly in patients with advanced age. Surgery is the primary treatment strategy. Intrahepatic and extrahepatic lesions, as well as dysplasia and invasive carcinoma have their own unique characteristics. The long-term prognosis of IPNB is generally poor.