Gigante Elia, Paradis Valérie, Ronot Maxime, Cauchy François, Soubrane Olivier, Ganne-Carrié Nathalie, Nault Jean-Charles
Service d'hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France.
Centre de recherche sur l'inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France.
JHEP Rep. 2020 Aug 24;3(1):100174. doi: 10.1016/j.jhepr.2020.100174. eCollection 2021 Feb.
Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations ( and fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers.
肝内胆管癌、纤维板层癌、肝血管内皮瘤和肝血管肉瘤占原发性肝癌的比例不到5%。纤维板层癌和肝血管内皮瘤分别由独特的体细胞基因改变（分别为 和 融合）驱动，而肝内胆管癌的发病机制更为复杂，其组织学多样性表明了这一点。组织学是诊断的金标准，即使在专家中心，由于这些肝癌的发病率较低，诊断仍具有挑战性。可行时，手术切除是治疗的基石，肝血管内皮瘤还可进行肝移植。局部区域治疗和全身治疗的作用仍研究不足。在本综述中，我们旨在描述这些罕见原发性肝癌在诊断和临床管理方面的最新进展。
