Center for Children's Cancer Research at Huntsman Cancer Institute, Department of Oncological Sciences, University of Utah School of Medicine, Salt Lake City, Utah 84112, USA.
Annu Rev Pathol. 2012;7:145-59. doi: 10.1146/annurev-pathol-011110-130237. Epub 2011 Sep 19.
Approximately one-third of sarcomas contain specific translocations. Ewing sarcoma is the prototypical member of this group of sarcomas; it was the first to be recognized pathologically as a singular entity and to have its signature translocation defined cytogenetically, which led to the identification of its key driver alteration, the EWS-FLI1 gene fusion that encodes this aberrant, chimeric transcription factor. We review recent progress in selected areas of Ewing sarcoma research, including the application of genome-wide chromatin immunoprecipitation analyses, to provide a comprehensive view of the EWS-FLI1 target gene repertoire, the identification of EWS-FLI1 target genes that may also point to therapeutically targetable pathways, and data from model systems as they relate to the elusive cell of origin of Ewing sarcoma and its possible similarities to mesenchymal stem cells.
大约三分之一的肉瘤含有特定的易位。尤文肉瘤是该组肉瘤的典型成员；它是第一个被病理上确认为单一实体的肉瘤，其特征性易位在细胞遗传学上被定义，这导致了其关键驱动改变的鉴定，即 EWS-FLI1 基因融合，该融合编码这种异常的嵌合转录因子。我们综述了尤文肉瘤研究选定领域的最新进展，包括全基因组染色质免疫沉淀分析的应用，以提供对 EWS-FLI1 靶基因谱的全面了解，鉴定可能也指向治疗靶点途径的 EWS-FLI1 靶基因，以及模型系统的数据，因为它们与尤文肉瘤起源细胞的难以捉摸及其与间充质干细胞的可能相似性有关。
