SOLID VARIANT ALVEOLAR RHABDOMYOSARCOMA - A RHABDOMYOSARCOMA WITH POOR PROGNOSIS

复习原发肿瘤的组织学切片 42例在NCI接受横纹肌肉瘤(RMS)方案治疗的患者 遇到15例软组织原始圆形细胞肉瘤 明显的模式，但细胞学特征类似于 肺泡RMS。这些病例的参考诊断为RMS-NOS， 胚胎性RMS或软组织原始肉瘤。所有这些案件 临床表现与肺泡型RMS相似。为所有人 基于上述原因，我们将RMS的这个原始子类型称为“固体变种”。 肺泡RMS。 评价电子显微镜(EM)或免疫细胞化学在临床中的应用 为了将这种原始的RMS与其他肿瘤区分开来，我们回顾了 15例中有13例采用了电子显微镜(EM)指纹 14例肌肉标记物的免疫细胞化学检测。记号笔 研究对象为骨骼肌肌球蛋白(MYS)、肌红蛋白(NMG)、 肌酸磷酸激酶(CPK)MM和结蛋白。结果显示，其中13人 15例符合RMS诊断标准。其他2个 应归类为原始肉瘤NOS。7例患者的细胞中有 Z带材料和/或基板和3抑制相当数量的 细胞质细丝。在所有的RMS中都有2到4个肌肉标志物 案子。Desmin是所有病例中存在的最可靠的单一标记物。 这些免疫细胞化学结果代表了我们先前工作的扩展 并支持实变型肺泡型RMS的肌源性本质。 他们还表明，EM和免疫细胞化学是有用的工具 在可疑病例中确认RMS的诊断。

Upon reviewing the histologic sections from the primary tumors of 42\patients treated in the rhabdomyosarcoma (RMS) protocol at the NCI, we encountered 15 primitive round cell sarcomas of the soft tissue with no overt pattern, but with cytologic characteristics resembling those of an alveolar RMS. The referring diagnoses on those cases were RMS NOS, embryonal RMS or primitive sarcoma of soft tissues. All these cases clinically behaved in a fashion similar to that of alveolar RMS. For all the above reasons, we termed this primitive subtype of RMS, "solid variant" alveolar RMS. To evaluate the use of electron microscopy (EM) or immunocytochemistry in distinguishing this primitive RMS from other tumors, we reviewed the electron microscopic (EM) prints on 13 out of 15 cases and employed immunocytochemistry for muscle markers on 14 cases. The markers investigated were skeletal muscle myosin (MYS), myoglobin (NMG), creatine-phosphokinase (CPK) MM and desmin. The results showed that 13 out of 15 cases met the criteria for being diagnosed as RMS. The other 2 should be classified as primitive sarcomas NOS. Seven cases had cells with Z-band material and/or basal lamina and 3 inhibited a fair amount of cytoplasmic filaments. Two to 4 muscle markers were present in all RMS cases. Desmin was the most reliable single marker present in all cases. These immunocytochemical results represent extension of our previous work on RMS and support the myogenous nature of the solid variant alveolar RMS. They also suggest that EM and immunocytochemistry are useful tools to confirm the diagnosis of RMS in doubtful cases.