Lauterio Andrea, De Carlis Riccardo, Centonze Leonardo, Buscemi Vincenzo, Incarbone Niccolò, Vella Ivan, De Carlis Luciano
Department of General Surgery and Transplantation, Niguarda Ca' Granda Hospital, 20162 Milan, Italy.
School of Medicine and Surgery, University of Milano-Bicocca, 20162 Milan, Italy.
Cancers (Basel). 2021 Jul 21;13(15):3657. doi: 10.3390/cancers13153657.
Cholangiocarcinoma accounts for approximately 10% of all hepatobiliary tumors and represents 3% of all new-diagnosed malignancies worldwide. Intrahepatic cholangiocarcinoma (i-CCA) accounts for 10% of all cases, perihilar (h-CCA) cholangiocarcinoma represents two-thirds of the cases, while distal cholangiocarcinoma accounts for the remaining quarter. Originally described by Klatskin in 1965, h-CCA represents one of the most challenging tumors for hepatobiliary surgeons, mainly because of the anatomical vascular relationships of the biliary confluence at the hepatic hilum. Surgery is the only curative option, with the goal of a radical, margin-negative (R0) tumor resection. Continuous efforts have been made by hepatobiliary surgeons in order to achieve R0 resections, leading to the progressive development of aggressive approaches that include extended hepatectomies, associating liver partition, and portal vein ligation for staged hepatectomy, pre-operative portal vein embolization, and vascular resections. i-CCA is an aggressive biliary cancer that arises from the biliary epithelium proximal to the second-degree bile ducts. The incidence of i-CCA is dramatically increasing worldwide, and surgical resection is the only potentially curative therapy. An aggressive surgical approach, including extended liver resection and vascular reconstruction, and a greater application of systemic therapy and locoregional treatments could lead to an increase in the resection rate and the overall survival in selected i-CCA patients. Improvements achieved over the last two decades and the encouraging results recently reported have led to liver transplantation now being considered an appropriate indication for CCA patients.
胆管癌约占所有肝胆肿瘤的10%，占全球所有新诊断恶性肿瘤的3%。肝内胆管癌（i-CCA）占所有病例的10%，肝门部（h-CCA）胆管癌占病例的三分之二，而远端胆管癌占其余四分之一。h-CCA最早由Klatskin于1965年描述，是肝胆外科医生面临的最具挑战性的肿瘤之一，主要是因为肝门部胆管汇合处的解剖血管关系。手术是唯一的治愈选择，目标是进行根治性、切缘阴性（R0）的肿瘤切除。肝胆外科医生一直在不断努力以实现R0切除，从而促使包括扩大肝切除术、联合肝分割和门静脉结扎分期肝切除术、术前门静脉栓塞以及血管切除等积极手术方式的逐步发展。i-CCA是一种侵袭性胆管癌，起源于二级胆管近端的胆管上皮。i-CCA在全球的发病率正在急剧上升，手术切除是唯一可能治愈的治疗方法。积极的手术方式，包括扩大肝切除和血管重建，以及更多地应用全身治疗和局部区域治疗，可能会提高特定i-CCA患者的切除率和总生存率。过去二十年取得的进展以及最近报道的令人鼓舞的结果，使得肝移植现在被认为是CCA患者的一个合适适应证。
