Division of Oncology, Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Lancet Oncol. 2010 Feb;11(2):184-92. doi: 10.1016/S1470-2045(09)70286-4.
Progress in the treatment of Ewing's sarcoma, the second most common bone tumour in children and adolescents, has improved survival from about 10% in the period before chemotherapy was introduced to about 75% today for patients with localised tumours. However, patients with metastases still fare badly, and the therapy carries short-term and long-term toxicities. Multidisciplinary care is indispensable for these patients. Molecular techniques and new imaging modalities are affecting the diagnosis and classification of patients with Ewing's sarcoma. Cooperative group studies have led to chemotherapy regimens using the same drugs (vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide), although the exact regimens differ in Europe and North America. The EWS-ETS family of gene fusions and their downstream effects in Ewing's sarcomas provide opportunities for new approaches to treatment. These include the inhibition of the fusion gene or its protein product, and pathways related to IGF1 and mTOR. Inhibition of tyrosine kinases, exploitation of non-apoptotic cell death, and interference with angiogenesis are promising new approaches. With many new approaches and relatively few patients, it will be challenging to integrate new and established treatments through clinical trials.
尤因肉瘤（Ewings sarcoma）是儿童和青少年中第二常见的骨肿瘤，在化疗引入之前，局部肿瘤患者的生存率约为 10%，而现在已经提高到约 75%。然而，转移性患者的预后仍然很差，而且治疗存在短期和长期毒性。对于这些患者，多学科护理是必不可少的。分子技术和新的成像方式正在影响尤因肉瘤患者的诊断和分类。协作组研究导致了使用相同药物（长春新碱、阿霉素、环磷酰胺、异环磷酰胺和依托泊苷）的化疗方案，尽管欧洲和北美的具体方案有所不同。EWS-ETS 基因融合及其在尤因肉瘤中的下游效应为治疗提供了新的方法。这些方法包括抑制融合基因或其蛋白产物，以及与 IGF1 和 mTOR 相关的途径。抑制酪氨酸激酶、利用非凋亡性细胞死亡和干扰血管生成是很有前途的新方法。有许多新的方法，而患者相对较少，通过临床试验整合新的和已确立的治疗方法将具有挑战性。
