近年来，由于我们对肝癌发生机制的认识不断进步，罕见的原发性肝癌（PLC），包括肝细胞胆管癌、纤维板层癌和肝上皮样血管内皮瘤越来越受到人们的关注。在这篇立场文件中，代表肿瘤学、肝病学、病理学、放射学、外科和分子生物学的国际专家小组总结了有关罕见 PLC 的发病机制、诊断和治疗的现有信息和证据。虽然一些罕见 PLC 的全身治疗临床试验正在进行中，但显然需要更多涉及国内和国际合作的研究。版权所有 © 2024。由 Elsevier B.V. 出版。

In recent years, owing to advances in our understanding of hepatocarcinogenesis, rare primary liver cancers (PLCs), including combined hepatocellular-cholangiocarcinoma, fibrolamellar carcinoma, and hepatic epithelioid hemangioendothelioma have garnered increased attention. In this position paper, an international panel of experts representing oncology, hepatology, pathology, radiology, surgery, and molecular biology has summarised the available information and evidence on the pathogenesis, diagnosis, and treatment of rare PLCs. While clinical trials of systemic treatments are underway for some rare PLCs, it is evident that more research, involving national and international collaboration, is required.Copyright © 2024. Published by Elsevier B.V.