El-Diwany Ramy, Pawlik Timothy M, Ejaz Aslam
Department of Surgery, Johns Hopkins University, 600 N. Wolfe St, Tower 110 Baltimore, MD 21287, USA.
Department of Surgery, The Ohio State University, 320 W. 10th Avenue, M-260 Starling Loving Hall, Columbus, OH 43210, USA.
Surg Oncol Clin N Am. 2019 Oct;28(4):587-599. doi: 10.1016/j.soc.2019.06.002.
Intrahepatic cholangiocarcinoma (ICC) arises from the epithelial cells of the intrahepatic and extrahepatic bile ducts and occurs proximal to the segmental biliary ducts. Risk factors include chronic hepatitis and cirrhosis, biliary inflammatory diseases, and hepatobiliary flukes, although in most cases, no known risk factor is identified. ICC is highly aggressive, with long-term survival only observed in patients with a complete R0 surgical resection. Technical and physiologic resectability should be considered when performing an operative plan. Nodal involvement is among the most important prognostic factors associated with survival and a porta hepatis lymphadenectomy should be performed at the time of resection. Adjuvant chemotherapy can provide a significant survival benefit for patients with more advanced or aggressive tumors. Systemic, locoregional, and targeted therapies exist for patients with unresectable or metastatic disease.
肝内胆管癌（ICC）起源于肝内和肝外胆管的上皮细胞，发生于肝段胆管近端。危险因素包括慢性肝炎和肝硬化、胆道炎症性疾病以及肝吸虫，不过在大多数情况下，并未发现已知的危险因素。ICC具有高度侵袭性，只有在接受R0根治性手术切除的患者中才能观察到长期生存。制定手术方案时应考虑技术和生理可切除性。淋巴结受累是与生存相关的最重要预后因素之一，切除时应行肝门淋巴结清扫术。辅助化疗可为病情更晚期或侵袭性更强的肿瘤患者带来显著的生存获益。对于无法切除或发生转移的患者，存在全身治疗、局部区域治疗和靶向治疗。
