Barthère Xavier, Guillerm Sophie, Quero Laurent, Le Maignan Christine, Torossian Nourezza, Verillaud Benjamin, Itti Ramona, Hennequin Christophe
Radiation oncology department, groupe hospitalier Saint-Louis-Lariboisière, université Paris-VII, AP-HP, 75475 Paris, France.
Medical oncology department, groupe hospitalier Saint-Louis-Lariboisière, université Paris-VII, AP-HP, 75475 Paris, France.
Cancer Radiother. 2020 Dec;24(8):870-875. doi: 10.1016/j.canrad.2020.03.015. Epub 2020 Nov 7.
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.
肺泡横纹肌肉瘤（ARMS）是儿童最常见的软组织肉瘤，但在成人中很少见。大多数成人治疗方案是从儿童方案改编而来。在此，我们报告一例53岁女性，诊断为鼻肺泡横纹肌肉瘤，诊断时为IV期，接受化疗（一种源自儿童方案pEpSSG RMS 2005的方案），化疗后部分缓解，随后接受放化疗。我们对成人头颈部ARMS进行了系统回顾，共发现29例。应先采用不同方案（VAC、VAI或VIE）进行初始化疗，然后进行手术和/或外照射放疗（最好采用调强放疗）。外照射放疗似乎对每例ARMS都有益，常规分割剂量约为50Gy，最终对残留肿瘤进行加量照射。靶区体积必须在化疗前影像上确定。近距离放疗和质子治疗正在评估中。
