Calvisi Diego F, Solinas Antonio
Institute of Pathology, University of Regensburg, Regensburg, Germany.
Department of Biomedical Sciences, University of Sassari, Sassari, Italy.
Transl Gastroenterol Hepatol. 2020 Jul 5;5:42. doi: 10.21037/tgh.2019.12.03. eCollection 2020.
The survival rate for patients with metastatic hepatoblastoma (HB) is steadily increased in the last thirty years from 27% to 79%. These achievements result from accurate risk stratification and effective chemotherapy and surgical care. However, patients with poor prognosis require more effective therapies. Recent years have witnessed new insights on the biology of HB, setting the stage for molecular classification and new targets of therapy. We review here the molecular pathology of HB, focusing on the driver genes involved in the process of oncogenesis and the identification of novel targets. We also address the role of models in elucidating the mechanisms of development of this disease and the pre-clinical phase of new treatment modalities.
在过去三十年中，转移性肝母细胞瘤（HB）患者的生存率稳步提高，从27%升至79%。这些成就得益于准确的风险分层以及有效的化疗和手术治疗。然而，预后较差的患者需要更有效的治疗方法。近年来，人们对HB的生物学特性有了新的认识，为分子分类和新的治疗靶点奠定了基础。我们在此回顾HB的分子病理学，重点关注肿瘤发生过程中涉及的驱动基因以及新靶点的识别。我们还讨论了模型在阐明该疾病发展机制和新治疗模式临床前阶段中的作用。
