Newton W A, Soule E H, Hamoudi A B, Reiman H M, Shimada H, Beltangady M, Maurer H
Department of Laboratory Medicine, Children's Hospital, Columbus, OH 43205.
J Clin Oncol. 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67.
Histopathologic material from 1,782 patients registered in the Intergroup Rhabdomyosarcoma Study Committee (IRS)-I and -II were reviewed by the IRS Pathology Committee in order to provide a uniform approach to classification and correlate patient survival with tumor type. Categories considered eligible were the four types of rhabdomyosarcoma (RMS) (criteria of Horn and Enterline), extraosseous Ewing's tumor (EOE), and a group of somewhat variable undifferentiated sarcomas designated small round cell sarcoma, type indeterminate (STI). Tumors that were clearly sarcomas but were unclassifiable also were included (NOS). The committee diagnoses were embryonal (Emb) RMS in 877 (54%), alveolar (Alv) RMS in 343 (21%), botryoid (Botr) RMS in 88 (5%), pleomorphic (Pleo) RMS in 11 (1%), STI in 135 (8%), and EOE in 84 (5%). One in nine were mixtures of types, eg, Emb and Alv. Five percent of the sarcomas could not be classified because of inadequate material. In general, there was close agreement (94%) between the review committee and institutional pathologists in the diagnosis of RMS, but not in the specific types, particularly Alv RMS (41%) and STI (36%). This observation is important, since patients with Alv RMS and STI tumors had decreased survival compared with the other histologies. The prognosis varied by histology, with Botr having the best, Alv RMS and STI the worst, and Emb RMS and EOE an intermediate prognosis.
横纹肌肉瘤研究协作组（IRS）-I和-II登记的1782例患者的组织病理学材料由IRS病理学委员会进行了审查，以便提供统一的分类方法，并将患者生存率与肿瘤类型相关联。符合条件的类别包括四种类型的横纹肌肉瘤（RMS）（霍恩和恩特林标准）、骨外尤因肉瘤（EOE）以及一组有些多变的未分化肉瘤，称为小圆细胞肉瘤，类型不确定（STI）。明显为肉瘤但无法分类的肿瘤也包括在内（未分类，NOS）。委员会诊断为胚胎型（Emb）RMS的有877例（54%），腺泡型（Alv）RMS的有343例（21%），葡萄簇型（Botr）RMS的有88例（5%），多形型（Pleo）RMS的有11例（1%），STI的有135例（8%），EOE的有84例（5%）。九分之一为混合型，例如Emb和Alv。5%的肉瘤因材料不足无法分类。总体而言，审查委员会与机构病理学家在RMS诊断方面的一致性较高（94%），但在具体类型方面并非如此，尤其是Alv RMS（41%）和STI（36%）。这一观察结果很重要，因为与其他组织学类型相比，Alv RMS和STI肿瘤患者的生存率较低。预后因组织学类型而异，Botr预后最佳，Alv RMS和STI最差，Emb RMS和EOE预后中等。
