Qualman S J, Coffin C M, Newton W A, Hojo H, Triche T J, Parham D M, Crist W M
Department of Laboratory Medicine and IRSG Pathology Center, Children's Hospital and Ohio State University School of Medicine, 700 Children's Drive, Columbus, OH 43205, USA.
Pediatr Dev Pathol. 1998 Nov-Dec;1(6):550-61. doi: 10.1007/s100249900076.
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, and 75% of such cases in the United States are reviewed at the Pathology Center for the Intergroup Rhabdomyosarcoma Study Group (IRSG). The first four generations of IRSG therapeutic trials (IRS I-IV) and supportive pathologic studies have generated a new International Classification of Rhabdomyosarcoma (ICR) that offers new morphologic concepts to the practicing pathologist. The objective of this report is to clearly define emerging histopathologic categories of RMS as defined by the ICR, and to emphasize correlative immunohistochemical or molecular studies. Emerging ICR variants of RMS place the patient in widely divergent prognostic categories (superior, botryoid or spindle cell variants; poor, solid alveolar or diffusely anaplastic variants). The cardinal histopathologic features of the ICR combined with results of studies of fusion genes seen with t(1;13) and t(2;13) will help delineate therapeutic subgroups of RMS for the fifth generation (IRS V) of IRSG studies. Consequently, it is imperative for the practicing pathologist to be familiar with the practical workup and diagnosis of RMS in childhood.
横纹肌肉瘤（RMS）是儿童期最常见的软组织肉瘤，在美国，此类病例的75%由横纹肌肉瘤研究组（IRSG）病理中心进行评估。IRSG的前四代治疗试验（IRS I-IV）及相关病理研究产生了新的横纹肌肉瘤国际分类（ICR），为执业病理学家提供了新的形态学概念。本报告的目的是明确界定ICR所定义的RMS新的组织病理学类别，并强调相关的免疫组织化学或分子研究。RMS新出现的ICR变异型使患者处于预后差异很大的类别中（预后良好的葡萄状或梭形细胞变异型；预后不良的实性肺泡型或弥漫性间变型）。ICR的主要组织病理学特征与t(1;13)和t(2;13)融合基因的研究结果将有助于为IRSG第五代（IRS V）研究界定RMS的治疗亚组。因此，执业病理学家必须熟悉儿童RMS的实际检查和诊断。
