von Schweinitz Dietrich
Dr. von Hauner Children's Hospital, University of Munich, Munich, Germany.
Semin Pediatr Surg. 2012 Feb;21(1):21-30. doi: 10.1053/j.sempedsurg.2011.10.011.
Hepatoblastoma is the most common liver tumor of early childhood. According to recent studies its incidence seems to be increasing in North America and Europe. Since new histological variants have been described recently the formerly clear-cut distinction of hepatoblastoma and hepatocellular carcinoma may not be valid anymore and a new histological classification will be inaugurated by an international working group. Recent research identified prognostically relevant gene signatures as well as potential molecular targets for therapy of hepatoblastoma. The multicentric study groups in the USA, Europe and Japan recommend cisplatin based chemotherapy for neoadjuvant and adjuvant treatment. However, their risk stratification systems and general treatment strategies differ substantially. Therefore the four groups agreed to pool their patients' data for an analysis of prognostic criteria which can be used for defining common risk groups. While 90% of standard risk and 65% of high risk hepatoblastomas can be cured, the still dismal outcome of multifocal disseminated and metastasising tumors warrants the investigation of new cytotoxic drugs and substances against specific molecular targets.
肝母细胞瘤是儿童早期最常见的肝脏肿瘤。根据最近的研究，其发病率在北美和欧洲似乎呈上升趋势。由于最近描述了新的组织学变异型，以前肝母细胞瘤和肝细胞癌之间明确的区分可能不再有效，一个国际工作组将开创一种新的组织学分类方法。最近的研究确定了与肝母细胞瘤预后相关的基因特征以及潜在的分子治疗靶点。美国、欧洲和日本的多中心研究小组推荐以顺铂为基础的化疗用于新辅助和辅助治疗。然而，它们的风险分层系统和总体治疗策略有很大差异。因此，这四个小组同意汇总他们患者的数据，以分析可用于定义共同风险组的预后标准。虽然90%的标准风险肝母细胞瘤和65%的高风险肝母细胞瘤可以治愈，但多灶性播散性和转移性肿瘤仍然令人沮丧的预后结果促使人们研究针对特定分子靶点的新的细胞毒性药物和物质。
