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横纹肌肉瘤及相关肉瘤的分类。病理学方面及新分类的提议——一项横纹肌肉瘤协作组研究

Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study.

作者信息

Newton W A, Gehan E A, Webber B L, Marsden H B, van Unnik A J, Hamoudi A B, Tsokos M G, Shimada H, Harms D, Schmidt D

机构信息

Children's Cancer Group, Arcadia, California, USA.

出版信息

Cancer. 1995 Sep 15;76(6):1073-85. doi: 10.1002/1097-0142(19950915)76:6<1073::aid-cncr2820760624>3.0.co;2-l.

DOI:10.1002/1097-0142(19950915)76:6<1073::aid-cncr2820760624>3.0.co;2-l
PMID:8625211
Abstract

BACKGROUND

There is a need to develop a single prognostically significant classification of rhabdomyosarcomas (RMS) and other related tumors of children, adolescents, and young adults which would be a current guide for their diagnosis, allow valid comparison of outcomes between protocols carried out anywhere in the world, and should enhance recognition of prognostic subsets.

METHOD

Sixteen pathologists from eight pathology groups, representing six countries and several cooperative groups, classified by four histopathologic classification schemes 800 representative tumors of the 999 eligible cases treated on Intergroup Rhabdomyosarcoma Study II. Each tumor was classified according to each of the four systems by each of the pathologists. In addition, two independent subsamples of 200 of the 800 patients were reviewed according to the new system, so that 343 distinct patients were reviewed once, and 57 of these twice.

RESULTS

A study of the survival rates of all subtypes in the sample of 800 patients led to the formation of a new system. This was tested on two independent subsets of 200 of the original cases and found to be reproducible and predictive of outcome by univariate analysis. A multivariate analysis of the 343 patients classified according to the new system indicated that a survival model including pathologic classification and known prognostic factors of primary site, clinical group, and tumor size was significantly better at predicting survival than a model with only the known prognostic factors.

CONCLUSION

This new classification, termed International Classification of Rhabdomyosarcoma (ICR) by the authors, was reproducible and predictive of outcome among patients with differing histologies treated uniformly on the Intergroup Rhabdomyosarcoma II protocols. We believe it should be utilized by all pathologists and cooperative groups to classify rhabdomyosarcomas in order to provide comparability among and within multi-institutional studies.

摘要

背景

需要开发一种针对儿童、青少年和青年横纹肌肉瘤(RMS)及其他相关肿瘤的具有单一预后意义的分类方法,该方法应成为当前诊断的指南,允许对世界各地实施的方案之间的结果进行有效比较,并应增强对预后亚组的识别。

方法

来自八个病理组的16名病理学家,代表六个国家和几个合作组,根据四种组织病理学分类方案对国际横纹肌肉瘤研究II中治疗的999例合格病例中的800例代表性肿瘤进行分类。每位病理学家根据四种系统中的每一种对每个肿瘤进行分类。此外,根据新系统对800名患者中的200名患者的两个独立子样本进行了审查,因此343名不同的患者接受了一次审查,其中57名接受了两次审查。

结果

对800例患者样本中所有亚型的生存率进行研究后形成了一个新系统。该系统在原始病例的两个200例独立子集中进行了测试,发现通过单变量分析具有可重复性且可预测结果。对根据新系统分类的343名患者进行的多变量分析表明,一个包括病理分类以及原发部位、临床分组和肿瘤大小等已知预后因素的生存模型在预测生存方面明显优于仅包含已知预后因素的模型。

结论

作者将这种新分类称为国际横纹肌肉瘤分类(ICR),在国际横纹肌肉瘤II方案中接受统一治疗的不同组织学患者中具有可重复性且可预测结果。我们认为所有病理学家和合作组都应使用它来对横纹肌肉瘤进行分类,以便在多机构研究之间和内部提供可比性。

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