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尽管肝内胆管细胞癌(ICC)发病率较低,但它是继肝细胞癌后最常见的原发性肝脏恶性肿瘤,而且,近几十年来发病率及其病死率呈明显上升趋势.一方面,由于缺乏特征性的临床症状、特异性的肿瘤标志物以及影像学表现,早期诊断极为困难;另一方面,该肿瘤具有高度恶性的生物学行为,肝外转移早,即使手术切除,术后常常较早复发,患者总体预后极差.遗憾的是,由于其发病率较低,患病人数相对较少,临床上对其重视不够;由于临床资料、标本缺乏,对ICC的研究也不够深入,发病机制仍不清楚.现就近年来ICC的新进展进行探讨,主要包括发病危险因素、分子机制、新的诊断标志物、治疗(包括分子靶向药物)等,旨在提高人们对ICC的理解与认识.“,”Although intrahepatic cholangiocellular carcinoma (ICC) has a relatively low incidence rate,it ranks the second in the most common primary malignant liver ttumors,with hepatocellular carcinoma ranking the first.Meanwhile,its incidence and mortality rates tend to increase significantly in the past decades.On the one hand,due to a lack of characteristic clinical symptoms,specific tumor markers,and imaging findings,early diagnosis of ICC is extremely difficult;on the other hand,ICC has highly malignant biological behaviors and early extrahepatic metastasis,so patients often experience early recurrence even if surgical resection is performed,which leads to the poor prognosis of such patients.Unfortunately,since ICC has a low incidence rate and relatively few patients,it is not taken seriously in clinical practice.Since there is a lack of clinical data,specimens,and in-depth studies on ICC,its pathogenesis remains unclear.This article discusses recent advances in ICC,including risk factors,molecular mechanism,new diagnostic markers,and therapies (including molecular targeted drugs).