梭形细胞/硬化性横纹肌肉瘤7例临床病理分析  

Spindle cell/sclerosing rhabdomyosarcoma:a clinicopathological study of seven cases

作　　者：王安然 周军[1] 周露婷 樊月 郑赛芳 王朝夫[1] WANG An-ran;ZHOU Jun;ZHOU Lu-ting;FAN Yue;ZHENG Sai-fang;WANG Chao-fu(Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China)

机构地区：[1]上海交通大学医学院附属瑞金医院病理科,上海200025

出　　处：《临床与实验病理学杂志》2020年第11期1313-1316,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨梭形细胞/硬化性横纹肌肉瘤(spindle cell/sclerosing rhabdomyosarcoma,SRMS/ScRMS)的临床病理特点、诊断及鉴别诊断。方法分析7例SRMS的临床资料、组织学特征、免疫表型(采用免疫组化EnVision两步法染色),并复习相关文献。结果7例SRMS发生部位为左手掌、右小腿、左眼眶、会阴部、右肘部、腹膜后和左口腔。临床症状主要表现为肿物、疼痛和活动受限。影像学资料提示占位性病变。镜下见肿瘤由条束状排列的长梭形细胞组成,或含有大量嗜伊红色至嗜碱性的玻璃样或透明变性基质。免疫表型:瘤细胞均不同程度表达desmin(7/7)、MyoD1(6/7)、Myogenin(5/7)、SMA(3/7)、CK(AE1/AE3)(1/7),Caldesmon、EMA和S-100均不表达,Ki-67增殖指数5%~70%。治疗:患者均经历手术切除,3例化疗,1例放、化疗。患者随访2~59个月,2例原位复发,无死亡和转移。结论SRMS是横纹肌肉瘤的少见亚型,充分了解其临床病理学特征有助于鉴别其他梭形细胞肿瘤及以硬化性间质为特征的肿瘤,做出正确的诊断。Purpose To investigate the clinicopathological characteristics,diagnosis and differential diagnosis of spindle cell/sclerosing rhabdomyosarcoma(SRMS/ScRMS).Methods Immunohistochemical of EnVision staining was used.Seven cases of SRMS were retrospectively reviewed on the clinical data,histological and immunohistochemical features,the follow-up,and the related literature.Results The anatomic locations included the left palm,right crus,left orbit,perineum,right elbow,behind the peritoneal,and left cheek.The main clinical and radiographic manifestations were ache,space occupying lesion and limited mobility.Histologically,SRMS was comprised of cells arranged in cords and strands with elongated and fusiform nuclei or contained a large amount of eosinophilic to basophilic hyalinized matrix.Immunohistochemically,tumor cells were positive for desmin(7/7),and partly positive for MyoD1(6/7),Myogenin(5/7),SMA(3/7),CK(AE1/AE3)(1/7),but totally negative for Caldesmon,EMA and S-100.Proliferative index Ki-67 varied from 5%to 70%.During the follow-up period(2 to 59 months),none of patients was succumbed or adopted metastasis with two of them showing in situ recurrence.Conclusion SRMS is a rare subtype of rhabdomyosarcoma.A full understanding of its clinicopathological features is helpful to distinguish other spindle cell tumors and tumors characterized by sclerosing stroma,and to make a correct diagnosis.

关 键 词：梭形细胞横纹肌肉瘤 硬化性横纹肌肉瘤 病理学 诊断 鉴别诊断 免疫组织化学 

分 类 号：R738.6[医药卫生—肿瘤]