Hilar cholangiocarcinoma is one of the most difficult malignant tumors to treat in the biliary system. In Japan, 5-year survival rate of the disease has increased from 32.5% to 67.1% during the past 30 years. The impressive progress reflects the solid efforts in preoperative endoscopic diagnosis, innovation in surgery such as PTPE as well as hepato-pancreatoduodenectomy and perioperative treatment including replacement of the bile and synbiotic treatment, which have finally formed a set of standardized diagnosis and treatment systems. The present review intends to report the history, current status and remaining bottlenecks of the diagnosis and treatment system of hilar cholangiocarcinoma in Japan as follows.
肝门部胆管癌是胆道外科治疗难度最大的恶性肿瘤之一。在日本，过去的30余年间该疾病的5年生存率从32.5%升至67.1%。其背后反映出日本在术前的内镜诊断、包括门静脉栓塞术、半肝联合胰十二指肠切除术在内的术式创新以及包括胆汁还原、益生菌治疗等措施在内的围手术期支持治疗等诸多方面的坚实努力，并由此形成了一整套规范化的诊治体系。本文对该诊治体系的历史沿革、现状及尚存的问题进行简要介绍。.
Keywords: Bile duct neoplasms; Embolization, therapeutic; Hilar cholangiocarcinoma; Perioperative treatment.