Hilar cholangiocarcinoma: diagnosis, treatment options, and management.
Hilar cholangiocarcinoma: diagnosis, treatment options, and management.
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DOI：
10.3978/j.issn.2304-3881.2014.02.05
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发表时间：
2014-02-01
影响因子：
8
通讯作者：
Pawlik, Timothy M
中科院分区：
文献类型：
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作者：
Soares, Kevin C;Kamel, Ihab;Pawlik, Timothy M
肝门部胆管细胞癌(HC)是一种少见的疾病，预后较差，通常在6岁左右发病。在美国每年看到的3000例病例中，只有不到一半的肿瘤是可以切除的。多种危险因素与HC有关，最显著的是原发性硬化性胆管炎(PSC)、胆石病和寄生虫性肝病。患者通常表现为腹痛、瘙痒、体重减轻和黄疸。使用计算机断层扫描(CT)、磁共振成像(MRI)和超声(US)来确定胆道病变的特征。内窥镜逆行胆胰管造影术(ERCP)和经皮肝穿胆管造影术(PTC)评估肿瘤的局部导管范围，同时允许治疗性胆道引流。在确定肿瘤的解剖范围方面，MRCP显示了与PTC和ERCP相似的效果，并发症较少。治疗包括手术、放疗、化疗和光动力疗法。未来的残肝应该进行胆道引流，以降低胆红素水平，从而促进未来的肝脏肥大。标准治疗包括手术切缘阴性(R0)切除加肝外胆管切除、肝切除和全块淋巴清扫。不应进行局部切除。淋巴结侵犯、肿瘤分级和切缘阴性是重要的预后指标。在不可能根治性切除的情况下，肝移植在高度精选的患者中已显示出可接受的结果。尽管数据有限，但化疗适用于无法切除的肿瘤和功能状态良好的患者。肝细胞癌手术切除后的五年存活率在10%到40%之间，然而，即使在R0切除后，复发率也可以高达50%到70%。由于这种疾病的复杂性，建议对这种复杂的疾病采取多学科、多模式治疗的方法。
Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6(th) decade of life. Of the 3,000 cases seen annually in the United States, less than one half of these tumors are resectable. A variety of risk factors have been associated with HC, most notably primary sclerosing cholangitis (PSC), biliary stone disease and parasitic liver disease. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. Computed topography (CT), magnetic resonance imaging (MRI), and ultrasound (US) are used to characterize biliary lesions. Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) assess local ductal extent of the tumor while allowing for therapeutic biliary drainage. MRCP has demonstrated similar efficacies to PTC and ERCP in identifying anatomic extension of tumors with less complications. Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. Biliary drainage of the future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liver hypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. Local resection should not be undertaken. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40% however, recurrence can be as high as 50-70% even after R0 resection. Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease.