Abstract
Context .- Since 1995, the International Classification of Rhabdomyosarcoma has provided prognostically relevant classification for rhabdomyosarcoma (RMS) and allowed risk stratification for children with RMS. The International Classification of Rhabdomyosarcoma includes botryoid and spindle cell RMS as superior-risk groups, embryonal RMS as an intermediate-risk group, and alveolar RMS as an unfavorable-risk group. The 2013 World Health Organization (WHO) classification of skeletal muscle tumors modified the histologic classification of RMS to include sclerosing RMS as a type of spindle cell RMS separate from embryonal RMS. The current WHO classification includes embryonal, alveolar, spindle cell/sclerosing, and pleomorphic subtypes of RMS and does not separate the botryoid subtype. Objective .- To determine if the WHO classification applies to pediatric RMS. Design .- To accomplish this goal, we reviewed 9 consecutive Children's Oncology Group clinical trials to compare the WHO and International Classification of Rhabdomyosarcoma classifications with outcome and site of disease. Results .- Except for a subset of low-risk RMS, the outcome for botryoid was not significantly different from typical embryonal RMS when analyzed by primary site. Similarly, pediatric spindle cell and sclerosing patterns of RMS did not appear significantly different from typical embryonal RMS, with one exception: spindle cell RMS in the parameningeal region had an inferior outcome with 28% event-free survival. Conclusion .- Our data support use of the WHO RMS classification in the pediatric population, with the caveat that histologic diagnosis does not necessarily confer the same prognostic information in children as in adults.
摘要
背景:自1995年以来,横纹肌肉瘤国际分类提供了横纹肌肉瘤(RMS)预后相关性分类,并且可用于对儿童RMA患者进行危险分层。横纹肌肉瘤的国际分类包括葡萄状RMS和梭形细胞RMS(低危组)、胚胎性RMS(中危组)以及腺泡状RMS(高危组)。2013年世界卫生组织(WHO)骨骼肌肿瘤分类对RMS组织学分类进行了修改,将硬化性RMS作为梭形细胞RMS的一种类型从胚胎性RMS中分离出来。当前WHO分类中,RMS亚型包括胚胎性RMS、腺泡状RMS、梭形细胞/硬化性RMS以及多形性RMS,并且没有将葡萄状亚型单独列出。目的:明确WHO分类是否适用于儿童RMS。设计:为完成这一目标,我们连续回顾了9项儿童肿瘤小组临床试验,比较横纹肌肉瘤按照WHO分类和国际分类其预后和部位有无差异。结果:除了低危亚型RMS,当按原发部位分析RMS时,葡萄状RMS的预后与典型胚胎性RMS无显著差异。同样,儿童梭形细胞RMS和硬化性RMS与典型胚胎性RMS亦无显著差异,但一点除外:脑膜旁区域梭形细胞RMS预后较差,仅28%患者无病生存。结论:我们的数据支持儿童群体使用WHO RMS分类,但同时伴有一定条件,儿童群体组织学诊断并不能像成年人那样给予相同的预后信息。
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