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Abstract：
Alveolar rhabdomyosarcoma (ARMS) represents the most common childhood soft tissue sarcoma, but they are rarely seen among adults. Most of the protocols for adults are adapted from pediatric protocols. Here we report a case of a 53-year-old woman diagnosed with a nasal alveolar rhabdomyosarcoma, stage IV at diagnosis, treated by chemotherapy (a regimen inspired from the pediatric protocole pEpSSG RMS 2005) which led to partial response followed by chemo-radiotherapy. We performed a systematic review of adult head and neck ARMS and found 29 cases. Primary chemotherapy with different protocols (VAC, VAI or VIE) should be done followed by surgery and/or external beam radiotherapy (preferably with IMRT). EBRT seems beneficial to every ARMS with a dose around 50Gy in a conventional fractionation, eventually completed with a boost on residual tumor. The target volume must be defined on pre-chemotherapy imaging. Brachytherapy and proton therapy are under evaluation.
摘要：
肺泡横纹肌肉瘤（ARMS）是最常见的儿童软组织肉瘤，但在成年人中很少见。大多数成人协议是从儿科协议改编的。在这里，我们报告了一个53岁的女性被诊断患有鼻肺泡横纹肌肉瘤的病例，诊断时的IV期，通过化疗（该方案源自儿科方案pEpSSGRMS2005）治疗，导致部分反应，然后进行化疗。我们对成人头颈部ARMS进行了系统评价，发现29例。采用不同方案的主要化疗(VAC，应进行VAI或VIE），然后进行手术和/或外部束放疗（最好使用IMRT）。EBRT似乎对常规分级中剂量约为50Gy的每个ARMS都有益，最终完成了对残留肿瘤的加强。目标体积必须在化疗前成像时确定。近距离放射治疗和质子治疗正在评估中。
