横纹肌肉瘤（RMS）是儿童最常见的软组织肉瘤，恶性程度高，进展快。横纹肌肉瘤的病理类型、分期、患者年龄、分子学标记等是分层治疗的重要依据，与预后密切相关。目前，RMS经综合治疗，生存率已达70%以上；低危患者长期生存率已达90%，未来的研究将致力于减轻治疗强度，减少远期毒性；中危患者标准化学治疗方案仍然是VAC（环磷酰胺+长春新碱+更生霉素）方案。手术和放射治疗技术的改进有助于提高局部控制率和保存患者的器官功能。

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of children. It is a high-grade malignancy and aggressive. Factors, such as histological type, stage, age and molecular marker are important for stratification and treatment, even associated with the prognosis. Currently, with multidisciplinary treatment, patients with RMS have achieved a long-term survival rate of over 70%; the long-term survival rate of low-risk RMS is up to 90%; future studies should focus on decreasing the treatment intensity and long-term toxicity; standard chemotherapy regimen for intermediate-risk RMS is still VAC (Cyclophosphamide + Vincristine + Actinomycin ). The improvements of surgery and radiation therapy technology will help improve the local control rate and preservation of organ function in patients.