梭形细胞/硬化性横纹肌肉瘤9例临床病理研究

Clinicopathologic study of spindle cell/sclerosing rhabdomyosarcoma in nine cases

摘要:

目的：探讨2013版WHO新分类梭形细胞/硬化性横纹肌肉瘤的临床病理特征。方法：结合文献对福建医科大学附属第一医院病理科9例梭形细胞/硬化性横纹肌肉瘤临床、组织学形态及免疫组化结果进行分析。结果：梭形细胞/硬化性横纹肌肉瘤单独或混合存在，梭形细胞区呈漩涡状排列，免疫组化Desmin、MyoD1、Myogenin、CD56强表达，SMA、CD34、S-100弱表达；硬化区以玻璃样变的硬化基质为特点，可呈现小圆或梭形细胞形态，排列方式多样，核分裂数和KI-67阳性指数高于梭形细胞区。浅表部位梭形细胞横纹肌肉瘤预后最好，硬化性横纹肌肉瘤易复发或转移，核分裂计数及KI-67免疫组化对二者预后评估有帮助。结论：形态学梭形细胞/硬化性横纹肌肉瘤可混合存在，均表达横纹肌肉瘤特异性标记物，但硬化可能提示肿瘤侵袭性增强，预后不好。

Abstract:

Objective：To study the clinicopathologic characteristics of a new WHO subtype spindle cell/sclerosing type rhab－domyosarcoma. Methods：The clinicohistological and immunohistochemical features of 9 cases of spindle cell/sclerosing type rhab－domyosarcoma were studied and the literature was reviewed. Results：Spindle cell and sclerosing type rhabdomyosarcoma may exist independently or mix together. Spindle cell areas arranged in swirled pattern with strong expression of Desmin，MyoD1，Myogenin and CD56 and sometimes weak expression of CD34，S-100，SMA. Sclerosing type areas showed small round or spindle cells arranging in different patterns with prominent sclerosing matrix；nuclear fission count and KI-67 positive indexes of sclerosing type areas were higher than those of spindle cell areas. Superficial spindle cell rhabdomyosarcoma had favoured prognosis，while sclerosing type tended to relapse or metastasis；nuclear fission count and KI-67 indexes were helpful for prognosis. Conclusion：Spindle cell and sclerosing type rhabdomyosarcoma may exist together and express specific rhabdomyosarcoma markers；sclerosis may indicate aggressive behav－ior and worse prognosis.