文献类型：期刊文献

中文题名：1例成人大腿梭形细胞/硬化性横纹肌肉瘤的临床病理观察及文献复习

英文题名：Clinicopathological observation and literature review of a case of spindle cell/sclerosing rhabdomyosarcoma in the adult thigh

作者：刘伟[1,2];王佳乐[1];曾忆君[1];张登才[1,2]

第一作者：刘伟

机构：[1]甘肃中医药大学第一临床医学院,兰州730000;[2]甘肃省妇幼保健院(甘肃省中心医院)病理科,兰州730079

第一机构：甘肃中医药大学临床医学院

年份：2026

卷号：35

期号：1

起止页码：59

中文期刊名：中国组织化学与细胞化学杂志

外文期刊名：Chinese Journal of Histochemistry and Cytochemistry

基金：甘肃省自然科学基金(24JRRA618)。

语种：中文

中文关键词：梭形细胞;硬化性横纹肌肉瘤;病理学;诊断;鉴别诊断;免疫组织化学表型

外文关键词：Spindle cells;sclerosing rhabdomyosarcoma;pathology;diagnosis;differential diagnosis;immunohistochemistry

摘要：目的探讨成人梭形细胞/硬化性横纹肌肉瘤(spindle cell/sclerosing rhabdomyosarcoma,ScRMS/SRMS)的临床病理特征、诊断及鉴别诊断,以提高对该病的认识。方法分析1例SRMS/ScRMS患者的临床资料,观察其组织学形态及免疫表型,并复习相关文献。结果患者临床表现为左大腿皮下肿物伴疼痛及活动受限。影像学检查提示占位性病变。镜下肿瘤主要由条束状排列的长梭形细胞构成,间质伴有显著的嗜伊红玻璃样变或透明变性。免疫组织化学检测显示肿瘤细胞表达desmin、MyoD1、myogenin、SMA、CK(AE1/AE3)、SMARCA4及β-catenin;S100、SOX-10、H3K27M、CD34、STAT6、SS18-SSX、ALK、caldesmon均为阴性;Ki-67热点区增殖指数约80%。患者接受手术切除后辅以放化疗,术后3个月随访MRI提示原位复发。结论SRMS/ScRMS是一种罕见的横纹肌肉瘤亚型,掌握其独特的形态学及免疫表型特征有助于与其它梭形细胞肿瘤或伴有硬化性间质的肿瘤进行鉴别,从而为准确诊断及临床治疗提供依据。
Objective To investigate the clinicopathological features,diagnosis,and differential diagnosis of adult spindle cell/sclerosing rhabdomyosarcoma(ScRMS/SRMS)in order to enhance the understanding of this disease.Methods The clinical data of one patient with SRMS/ScRMS were analyzed,with observation of its histological morphology and immunophenotype,along with a review of relevant literature.Results The patient presented with a subcutaneous mass in the left thigh accompanied by pain and limited mobility.Imaging studies indicated a space-occupying lesion.Microscopically,the tumor was primarily composed of long spindle cells arranged in fascicles,with the stroma showing marked eosinophilic hyaline or glassy degeneration.Immunohistochemical analysis showed that the tumor cells were positive for desmin,MyoD1,myogenin,SMA,CK(AE1/AE3),SMARCA4,andβ-catenin;and negative for S100,SOX-10,H3K27M,CD34,STAT6,SS18-SSX,ALK,and caldesmon.The Ki-67 proliferation index was approximately 80%.The patient underwent surgical resection followed by adjuvant radiotherapy and chemotherapy.A follow-up MRI at 3 months postoperatively indicated local recurrence.Conclusion SRMS/ScRMS is a rare subtype of rhabdomyosarcoma.Familiarity with its distinct morphological and immunophenotypic characteristics is crucial for differentiating it from other spindle cell tumors or tumors with sclerosing stroma,thereby facilitating accurate diagnosis and guiding clinical management.