Background Combined hepatocellular-cholangiocarcinoma (cHCC-iCCA) is a rare type of primary liver cancer displaying characteristics of both hepatocytic and cholangiocytic differentiation. Summary Because of its aggressive nature, patients with cHCC-iCCA exhibit a poorer prognosis than those with HCC. Surgical resection and liver transplantation may be considered as curative treatment approaches; however, only a minority of patients are eligible at the time of diagnosis and postoperative recurrence rates are high. For cases that are not eligible for surgery, locoregional and systemic therapy are often administered based on treatment protocols applied for HCC or iCCA. Owing to the rarity of this cancer, there are still no established standard treatment protocols; therefore, the choice of therapy is often personalized and guided by the suspected predominant component. Further, the genomic and molecular heterogeneity of cHCC-iCCA can severely compromise the efficacy of the available therapies. Key Messages In the present review, we summarize the latest advances in cHCC-iCCA and attempt to clarify its terminology and molecular biology. We provide an overview of the etiology of cHCC-iCCA and present new insights into the molecular pathology of this disease that could contribute to further studies aiming to improve the patient outcomes through new systemic therapies.


中文翻译：

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混合性肝细胞胆管癌：生物学、诊断和治疗


背景 联合性肝细胞胆管癌（cHCC-iCCA）是一种罕见的原发性肝癌，表现出肝细胞和胆管细胞分化的特征。 概括 由于其侵袭性，cHCC-iCCA 患者的预后比 HCC 患者更差。手术切除和肝移植可被视为治愈性治疗方法；然而，只有少数患者在诊断时符合条件，术后复发率很高。对于不适合手术的病例，通常根据 HCC 或 iCCA 的治疗方案进行局部和全身治疗。由于这种癌症的罕见性，目前还没有既定的标准治疗方案；因此，治疗的选择通常是个性化的，并以可疑的主要成分为指导。此外，cHCC-iCCA 的基因组和分子异质性可能严重损害现有疗法的疗效。 关键信息 在本综述中，我们总结了 cHCC-iCCA 的最新进展，并试图阐明其术语和分子生物学。我们对 cHCC-iCCA 的病因学进行了概述，并对这种疾病的分子病理学提出了新的见解，这可能有助于进一步的研究，旨在通过新的全身疗法改善患者的预后。