Rudzinski Erin R
From Department of Laboratories, Seattle Children's Hospital, Seattle, WA.
Am Soc Clin Oncol Educ Book. 2013:425-8. doi: 10.14694/EdBook_AM.2013.33.425.
The International Classification of Rhabdomyosarcoma (ICR) has provided diagnostic criteria for rhabdomyosarcoma (RMS) and formed the basis of histologic risk stratification since its publication in 1995. However, the recognition of new variants of embryonal rhabdomyosarcoma (ERMS), shifts in the diagnostic criteria of alveolar rhabdomyosarcoma (ARMS), the increasing use of myogenin immunohistochemistry and recognition of the distinct biologic properties associated with fusion status all raised questions about the continued use of this classification system in the diagnosis and treatment of patients with RMS. Recent Children's Oncology Group Soft Tissue Sarcoma Committee analysis of histology and fusion status in the intermediate risk RMS study D9803 refined the histologic criteria of RMS. We validated the new diagnostic criteria against fusion status, allowing prospective examination of the prognostic value of histology compared with fusion status for risk-stratification of patients with RMS. This article summarizes the evolution of and current practices in the histologic and molecular classification of rhabdomyosarcoma.
国际横纹肌肉瘤分类（ICR）自1995年发布以来，为横纹肌肉瘤（RMS）提供了诊断标准，并构成了组织学风险分层的基础。然而，胚胎性横纹肌肉瘤（ERMS）新变种的发现、肺泡状横纹肌肉瘤（ARMS）诊断标准的变化、肌生成素免疫组织化学的日益广泛应用以及对与融合状态相关的独特生物学特性的认识，都引发了关于该分类系统在RMS患者诊断和治疗中是否仍应继续使用的疑问。儿童肿瘤学组软组织肉瘤委员会最近对中度风险RMS研究D9803中的组织学和融合状态进行的分析，完善了RMS的组织学标准。我们根据融合状态对新的诊断标准进行了验证，从而能够前瞻性地研究组织学与融合状态相比在RMS患者风险分层中的预后价值。本文总结了横纹肌肉瘤组织学和分子分类的演变及当前实践。
