Department of Laboratories, Seattle Children's Hospital, Seattle, Washington.
Department of Paediatric Histopathology, Royal Manchester Children's Hospital, Manchester Foundation Trust, Manchester, UK.
Pediatr Blood Cancer. 2021 Mar;68(3):e28798. doi: 10.1002/pbc.28798. Epub 2020 Dec 11.
The diagnosis and classification of rhabdomyosarcoma (RMS) has undergone several shifts over the last 30 years. While the main diagnostic categories remained the same, changes in the histologic criteria necessary for diagnosis, as well as varied reliance on immunohistochemical and molecular data over time, have created confusion, particularly regarding how these shifts impacted risk stratification and enrollment onto clinical trials. The goal of this report is to review the evolution and current status of RMS diagnosis, focusing on diagnostic criteria in the Children's Oncology Group (COG), the European Paediatric Soft Tissue Sarcoma Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). In addition, we emphasize research tools used to classify RMS and address biological questions within current clinical trials run by each group. The INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT) initiative will maximize potential to optimize risk stratification by recognizing and accounting for differences in historical data and current practices.
横纹肌肉瘤 (RMS) 的诊断和分类在过去 30 年中经历了几次转变。虽然主要的诊断类别保持不变，但诊断所需的组织学标准的变化，以及随着时间的推移对免疫组织化学和分子数据的不同依赖，造成了混淆，特别是这些变化如何影响风险分层和临床试验的入组。本报告的目的是回顾 RMS 诊断的演变和现状，重点介绍儿童肿瘤学组 (COG)、欧洲儿科软组织肉瘤组 (EpSSG) 和软组织肉瘤合作研究组 (CWS) 的诊断标准。此外，我们强调了用于分类 RMS 的研究工具，并解决了每个组当前临床试验中提出的生物学问题。国际软组织肉瘤协作组 (INSTRuCT) 倡议将通过认识和考虑历史数据和当前实践的差异，最大限度地提高优化风险分层的潜力。
