梭形细胞横纹肌肉瘤的临床病理特点

Clinicopathologic analysis of spindle cell rhabdomyosarcoma： report of 8 cases

目的研究梭形细胞横纹肌肉瘤（RMS）的临床病理特点及鉴别诊断。方法回顾性分析8例梭形细胞RMS患者的临床病理资料，对其临床表现、预后、组织形态和免疫组化结果进行分析。结果梭形细胞RMS可见于儿童和成年人，常发生于头颈部、宫颈和睾丸旁，多表现为无痛性肿块，其预后较差。梭形细胞RMS中瘤细胞多排列成束状、席纹状，其问常混杂着一些肥胖、短梭形或多角形、胞浆红染、核深染偏位的横纹肌母细胞。免疫组化结果为波形蛋白（vimentin）、肌调节蛋白（MyoD1）、结蛋白（desmin）、肌动蛋白（actin）和肌红蛋白（myoglobin）抗原阳性，S-100蛋白、甲胎蛋白（AFP）、胎盘碱性磷酸酶（PLAP）、角蛋白（CK）和c-kit蛋白（CD117）抗原阴性。结论梭形细胞RMS是一种少见的RMS，预后较差，肌源性标记（desmin）与MyoD1和myogenin的联合应用，有助于RMs的诊断及鉴别诊断。

Objective To study the clinicopathological features of spindle cell rhabdomyosarcoma （SCRMS） in order to differentiate it from other myosarcomas. Methods The clinical features, morphologic and immunohistochemical phenotypes of 8 SCRMSs were analyzed. Results SCRMS cells were found to be arranged in a fascicular or storiform pattern, in which a number of enlarged plump or polygonal shaped rhabdomyoblasts containing abundant eosinophilic cytoplasm with eccentrically placed enlarged hyperchromatic nuclei were mixed. Immunohistochemical staining results showed that vimentin, MyoD1, desmin,actin,myoglobin were positive in tumor cells, but S-100, plap, AE1/AE3, CK,CDll7 negative. The follow-up data showed that four ＇cases had died of the recurrent disease, one still alive and the remain three patients lost follow-up. Conclusion Spindle cell rhabdomyosarcoma is a rare embryonal rhabdomyosarcoma which occurs in the childhood or adulthood with a poor prognosis, and is frequently presented as a painless mass most frequently involveing the head and neck or cervical area or para-testis site. A combination of MyoD1, desmin and myoglobin immunohistochemical staining is helpful in differential diagnosis.