肝内胆管细胞癌（iCCA）起源于胆小管到二级胆管，是仅次于肝细胞癌的第二种最常见的原发性肝癌，不幸的是其发病率正在全球范围内增加。 由于其不显著的表现、晚期诊断、高度侵袭性和对治疗的耐受性，导致死亡率令人震惊。早期诊断、分子特征、准确分期和个体化的多学科治疗是研究人员和医生面临的挑战。不幸的是，iCCA在临床、基因组、表观遗传和分子水平上的高度异质性往往阻碍成功的治疗。尽管如此，近年来，在分子特征、手术治疗和靶向治疗方面取得了进展。最新的进展，以及认识到iCCA在CCA家族中代表着独特的实体，促使国际专家起草专门指南，以帮助医生实现基于证据的方法来管理iCCA的诊断、预后和治疗。版权所有©2023年欧洲肝脏研究协会。由Elsevier B.V.出版。保留所有权利。

Intrahepatic cholangiocarcinoma (iCCA) emerges inside the liver from bile ductules to the second-order bile ducts, represents the second most frequent primary liver cancer besides hepatocellular carcinoma and, unfortunately, is globally increasing in term of incidence. The silent presentation, late diagnosis, the highly aggressive nature and resistance to treatments lead to alarming mortality. Early diagnosis, molecular characterization, accurate staging and personalized multidisciplinary treatments represent challenges for researchers and physicians. Unfortunately, these challenges are beset by the high heterogeneity of iCCA at the clinical, genomic, epigenetic and molecular levels that very often preclude successful management. Nonetheless, in the last few years, progress has been achieved in molecular characterization, surgical management and targeted therapies. The recent advance together with the awareness that iCCA represents a distinct entity amongst the CCA family, led the ILCA and EASL governing boards to commission and charge international experts to draft dedicated guidelines to aid physicians towards an evidence-based approach for the diagnostic, prognostic, and therapeutic management of iCCA.Copyright © 2023 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.