A Contemporary Approach to Diagnosis and Treatment of Combined Hepatocellular-Cholangiocarcinoma.
A Contemporary Approach to Diagnosis and Treatment of Combined Hepatocellular-Cholangiocarcinoma.
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DOI：
10.1007/s11901-020-00556-4
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发表时间：
2020-12
期刊：
影响因子：
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通讯作者：
Razumilava N
中科院分区：
文献类型：
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作者：
Raevskaya O;Appelman H;Razumilava N
提供有关联合肝细胞胆管细胞癌的术语、流行病学、诊断和治疗的最新信息。CHCC-CCA是指在同一结节中含有不同程度的肝细胞癌和CCA成分并带有过渡区的肿瘤。CHCC-CCA发生在类似于肝硬变和非肝硬变的肝脏中，并与不良预后相关。TP53、TERT启动子和ARID1A突变是CHCC-CCA中最常见的遗传异常。融合基因PTMS-AP1G1是CHCC-CCA所特有的。诊断时需要做活检。手术切除仍然是治疗的首选，而肝移植治疗早期CHCC-CCA与良好的结果相关。以吉西他滨为基础的治疗对晚期CHCC-CCA有好处。CHCC-CCA是一组具有独特生物学行为的异质性原发性肝癌。需要进行多中心研究，以进行分子分析，为新的治疗方法提供信息，并了解肝移植、肝定向和靶向治疗这种罕见的侵袭性癌症的流行病学和益处。
To provide updates on terminology, epidemiology, diagnosis, and treatment of combined hepatocellular-cholangiocarcinoma (cHCC-CCA). cHCC-CCAs are tumors that in the same nodule contain a variable degree of HCC and CCA components with a transition zone. cHCC-CCAs develop in cirrhotic and non-cirrhotic livers like and is associated with poor outcomes. Mutations in TP53, TERT promoter, and ARID1A are the most common genetic aberrations in cHCC-CCA. Fusion gene PTMS-AP1G1 is unique for cHCC-CCA. A biopsy is required for diagnosis. Surgical resection remains treatment of choice, while liver transplantation for early cHCC-CCA is associated with favorable outcomes. Gemcitabine-based therapy shows benefits for advanced cHCC-CCA. cHCC-CCAs are a heterogeneous group of primary liver cancers with unique biological behavior. Multicenter studies are required for a molecular analysis to inform novel therapeutic approaches, and understand epidemiology and benefits of liver transplantation, liver-directed and targeted therapies for this rare aggressive cancer.