Rodrigues Pedro M, Olaizola Paula, Paiva Nuno A, Olaizola Irene, Agirre-Lizaso Alona, Landa Ana, Bujanda Luis, Perugorria Maria J, Banales Jesus M
Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute, Donostia University Hospital, University of the Basque Country (UPV/EHU), 20014 San Sebastian, Spain; email:
National Institute for the Study of Liver and Gastrointestinal Diseases, CIBERehd, Instituto de Salud Carlos III, 28029 Madrid, Spain.
Annu Rev Pathol. 2021 Jan 24;16:433-463. doi: 10.1146/annurev-pathol-030220-020455. Epub 2020 Dec 2.
Cholangiocarcinoma (CCA) encompasses a group of malignancies that can arise at any point in the biliary tree. Although considered a rare cancer, the incidence of CCA is increasing globally. The silent and asymptomatic nature of these tumors, particularly in their early stages, in combination with their high aggressiveness, intra- and intertumor heterogeneity, and chemoresistance, significantly compromises the efficacy of current therapeutic options, contributing to a dismal prognosis. During the last few years, increasing efforts have been made to unveil the etiologies and pathogenesis of these tumors and to develop more effective therapies. In this review, we summarize current findings in the field of CCA, mainly focusing on the mechanisms of pathogenesis, cells of origin, genomic and epigenetic abnormalities, molecular alterations, chemoresistance, and therapies.
胆管癌（CCA）是一组可发生于胆管树任何部位的恶性肿瘤。尽管被认为是一种罕见癌症，但CCA的发病率在全球范围内正在上升。这些肿瘤具有隐匿性和无症状性，尤其是在早期阶段，再加上其高侵袭性、肿瘤内和肿瘤间的异质性以及化疗耐药性，严重影响了当前治疗方案的疗效，导致预后不佳。在过去几年中，人们加大了对这些肿瘤的病因和发病机制的研究力度，并致力于开发更有效的治疗方法。在本综述中，我们总结了CCA领域的当前研究结果，主要关注发病机制、起源细胞、基因组和表观遗传异常、分子改变、化疗耐药性以及治疗方法。
