Department of Surgery, Sidney Kimmel Cancer Center, Johns Hopkins University School of Medicine, Harvey 611, 600 N Wolfe Street, Baltimore, MD 21287, USA.
Expert Rev Gastroenterol Hepatol. 2012 Aug;6(4):481-95. doi: 10.1586/egh.12.20.
Perihilar cholangiocarcinoma (CCA) is the second most common primary malignant tumor of the liver. In the USA, there are approximately 3000 cases of CCA diagnosed annually, with approximately 50-70% of these tumors arising at the hilar plate of the biliary tree. Risk factors include advanced age, male gender, primary sclerosing cholangitis, choledochal cysts, cholelithiasis, parasitic infection, inflammatory bowel disease, cirrhosis and chronic pancreatitis. Patients typically present with jaundice, abdominal pain, pruritus and weight loss. The mainstays of treatment include surgery, chemotherapy, radiation therapy and photodynamic therapy. Specific preoperative interventions for patients with perihilar CCA include endoscopic retrograde cholangiopancreatography, percutanteous transhepatic cholangiography and portal vein embolization. Surgical resection offers the only chance for curative therapy in perihilar CCA. R0 resection is of utmost importance and has been linked to improved survival. Major hepatic resection is needed to achieve both longitudinal and radial margins negative for tumor. Fractionated stereotactic body radiotherapy has shown promising results in CCA. Perihilar CCA typically presents with advanced disease, and many patients receive systemic therapy; however, the response to current regimens is limited. Orthotopic liver transplantation offers complete resection of locally advanced tumors in select patient groups.
肝门部胆管癌（CCA）是肝脏第二大常见原发性恶性肿瘤。在美国，每年大约有 3000 例 CCA 被诊断出来，其中约 50-70%的肿瘤发生在胆管树的胆门部位。危险因素包括年龄较大、男性、原发性硬化性胆管炎、胆总管囊肿、胆石症、寄生虫感染、炎症性肠病、肝硬化和慢性胰腺炎。患者通常表现为黄疸、腹痛、瘙痒和体重减轻。治疗的主要方法包括手术、化疗、放疗和光动力疗法。肝门部 CCA 患者的主要术前干预措施包括内镜逆行胰胆管造影术、经皮经肝胆管造影术和门静脉栓塞术。手术切除是肝门部 CCA 根治性治疗的唯一机会。R0 切除至关重要，已与改善生存相关联。需要进行大肝切除术以获得肿瘤阴性的纵向和径向切缘。分次立体定向体部放疗在 CCA 中显示出良好的结果。肝门部 CCA 通常表现为晚期疾病，许多患者接受系统治疗；然而，目前的治疗方案反应有限。在某些特定患者群体中，原位肝移植为局部晚期肿瘤提供了完全切除的机会。
