Hosoi Hajime, Tsuchiya Kunihiko, Sugimoto Tohru
Dept. of Pediatrics, Kyoto Prefectural University of Medicine.
Gan To Kagaku Ryoho. 2007 Feb;34(2):181-6.
Rhabdomyosarcoma (RMS) is the most common type of soft-tissue sarcoma in childhood. In Europe and the United States, survival rates of RMS patients, especially those with local or regional RMS, have dramatically improved from around 25% in 1970 to over 70% in 2001 as a result of the introduction of multimodality therapy and cooperative group trials. RMS patients require multimodal therapy including appropriate surgery at the primary site, histological diagnosis supported by molecular genetics, and a combination of chemotherapy and radiation therapy based on their risk group. In Japan, no nationwide studies on RMS have been performed so far. An analysis of registration data from the Japanese Society for Pediatric Surgery as well as our recent retrospective study indicates that the survival rates of each risk group are all lower than those in the US or Europe, even in the "low-risk" group. We have recently established the Japan Rhabdomyosarcoma Study Group (JRSG) and continued several studies in each risk group to improve the outcome and to establish a standard therapy in Japan.
横纹肌肉瘤(RMS)是儿童期最常见的软组织肉瘤类型。在欧洲和美国,由于采用了多模式治疗和合作组试验,RMS患者的生存率,尤其是局部或区域RMS患者的生存率,已从1970年的约25%显著提高到2001年的70%以上。RMS患者需要多模式治疗,包括在原发部位进行适当的手术、分子遗传学支持的组织学诊断,以及根据其风险组进行化疗和放疗的联合治疗。在日本,迄今为止尚未进行全国性的RMS研究。对日本小儿外科学会登记数据的分析以及我们最近的回顾性研究表明,即使在“低风险”组中,各风险组的生存率均低于美国或欧洲。我们最近成立了日本横纹肌肉瘤研究组(JRSG),并在每个风险组中继续进行多项研究,以改善治疗效果并在日本建立标准治疗方法。