Schürch W, Bochaton-Piallat M L, Geinoz A, d'Amore E, Laurini R N, Cintorino M, Bégin L R, Boivin Y, Gabbiani G
Department of Pathology, Hôtel-Dieu Hospital, St-Urbain, Quebec, Canada.
Am J Pathol. 1994 Apr;144(4):836-46.
Eleven human primary rhabdomyosarcomas (RMSs), including all histological variants, were analyzed morphologically, immunohistochemically for intermediate filament proteins and actin isoforms, and by means of Northern blots with probes specific for total actin, alpha-skeletal (SK), alpha-cardiac (CARD), and alpha-smooth muscle actin messenger (m)RNAs. All tumors disclosed ultrastructural evidence of skeletal muscle features with terminal differentiation in three cases. The RMSs contained immunohistochemically the intermediate filament proteins vimentin and desmin and reacted positively with the alpha-sarcomeric actin antibody, which recognizes alpha-SK and alpha-CARD actin isoforms. All RMSs reacted with the total actin probe, recognizing at 2.1 kb cytoplasmic actin mRNAs and at 1.7 kb alpha-actin mRNAs. With the specific probes, all RMSs expressed alpha-CARD actin mRNA, four neoplasms expressed also alpha-smooth muscle actin mRNA, whereas the probe for alpha-SK actin mRNA never produced a signal except in one case, in which the tumor masses were intermingled with non-neoplastic preexistent striated muscle fibers. Because alpha-CARD and alpha-smooth muscle actins are transiently expressed during normal skeletal muscle development, RMSs seem to follow normal skeletal myogenesis without completing the final step, consisting of alpha-SK actin mRNA expression. The use of Northern blots for alpha-CARD actin as an adjunct to conventional techniques may be helpful for the precise identification of primary RMSs compared to other soft tissue neoplasms.
对11例人类原发性横纹肌肉瘤(RMS)进行了分析,这些病例包括所有组织学变体,从形态学、免疫组织化学方面检测中间丝蛋白和肌动蛋白异构体,并通过Northern印迹法,使用针对总肌动蛋白、α-骨骼肌(SK)、α-心肌(CARD)和平滑肌α-肌动蛋白信使核糖核酸(mRNA)的特异性探针进行检测。所有肿瘤均显示出骨骼肌特征的超微结构证据,其中3例有终末分化。这些RMS在免疫组织化学上含有中间丝蛋白波形蛋白和结蛋白,并与α-肌节肌动蛋白抗体呈阳性反应,该抗体可识别α-SK和α-CARD肌动蛋白异构体。所有RMS均与总肌动蛋白探针反应,识别出2.1 kb的细胞质肌动蛋白mRNA和1.7 kb的α-肌动蛋白mRNA。使用特异性探针时,所有RMS均表达α-CARD肌动蛋白mRNA,4种肿瘤还表达α-平滑肌肌动蛋白mRNA,而α-SK肌动蛋白mRNA探针除1例肿瘤肿块与先前存在的非肿瘤性横纹肌纤维混合的病例外,从未产生信号。由于α-CARD和平滑肌α-肌动蛋白在正常骨骼肌发育过程中短暂表达,RMS似乎遵循正常骨骼肌生成过程,但未完成最后一步,即α-SK肌动蛋白mRNA表达。与其他软组织肿瘤相比,使用α-CARD肌动蛋白的Northern印迹法作为传统技术的辅助手段,可能有助于准确识别原发性RMS。