Several recent advances have been made in the diagnosis and therapy of malignant small round cell tumors that affect children, particularly in rhabdomyosarcoma, Ewing sarcoma, and other round cell sarcomas. These advances have provided new insights into the pathologic, histologic, and genomic characterization of specific tumor subtypes, which has led to the identification of novel therapeutic targets and improved stratification of risk. This has, in turn, led to improved efficacy in clinical trials of new drug combinations, thereby increasing the survival of patients with newly diagnosed and refractory or recurrent round cell sarcomas. Here, we review the progress that has been made using genomics to identify novel pathologic genomic rearrangements, as well as therapeutic targets. We also describe how clinical and molecular factors have helped refine risk stratification and therapies that have led to improved clinical outcomes in patients with round cell sarcomas.

中文翻译：

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横纹肌肉瘤、尤文肉瘤和其他圆形细胞肉瘤

最近在影响儿童的恶性小圆形细胞肿瘤的诊断和治疗方面取得了一些进展，特别是横纹肌肉瘤、尤文肉瘤和其他圆形细胞肉瘤。这些进展为特定肿瘤亚型的病理学、组织学和基因组特征提供了新的见解，从而确定了新的治疗靶点并改进了风险分层。这反过来又提高了新药组合临床试验的疗效，从而提高了新诊断的难治性或复发性圆细胞肉瘤患者的生存率。在这里，我们回顾了使用基因组学来识别新的病理基因组重排以及治疗靶点所取得的进展。