Malhi Harmeet, Gores Gregory J
Mayo Clinic College of Medicine, Rochester, MN, USA.
J Hepatol. 2006 Dec;45(6):856-67. doi: 10.1016/j.jhep.2006.09.001. Epub 2006 Sep 25.
Cholangiocarcinomas are tumors that arise anywhere in the biliary tract, presumably of cholangiocyte origin. The global incidence of this rare disease is on the rise. Several known risk factors exist, and link chronic biliary inflammation to the pathogenesis of cholangiocarcinoma. Among these, amplification of the epidermal growth factor receptor, the interleukin-6 signaling pathway, inducible nitric oxide, erb-2, and cyclooxygenase-2 are well defined. Most patients present late, with a median survival of months. Although, imaging studies and clinical context often indicate cholangiocarcinoma, pathologic and cytologic diagnosis is difficult to obtain. Advanced cytologic tests with fluorescence in situ hybridization or digital image analysis can increase diagnostic sensitivity. Surgical resection is the current therapy of choice for both intrahepatic and ductal cholangiocarcinomas. However, the 5-year survival is poor, with 60 to greater than 90% recurrence rates. In a single center experience, liver transplantation with neoadjuvant chemoirradiation, for highly selected patients, has a 5-year disease free survival of greater than 80%. Future targeted therapies will depend on a better understanding of the cellular and molecular biology of cholangiocarcinomas.
胆管癌是一种起源于胆管细胞、可发生于胆道任何部位的肿瘤。这种罕见疾病的全球发病率正在上升。已知有几种危险因素，它们将慢性胆管炎症与胆管癌的发病机制联系起来。其中，表皮生长因子受体、白细胞介素-6信号通路、诱导型一氧化氮、erb-2和环氧化酶-2的扩增已得到明确。大多数患者就诊时已处于疾病晚期，中位生存期仅数月。尽管影像学检查和临床情况常提示胆管癌，但病理和细胞学诊断却很难获得。采用荧光原位杂交或数字图像分析的先进细胞学检测可提高诊断敏感性。手术切除是目前肝内胆管癌和肝外胆管癌的首选治疗方法。然而，5年生存率较低，复发率为60%至90%以上。在一项单中心经验中，对于经过严格筛选的患者，新辅助放化疗后的肝移植5年无病生存率超过80%。未来的靶向治疗将依赖于对胆管癌细胞和分子生物学的更深入了解。
