摘要
目的:探讨非典型性骨外尤文肉瘤的临床病理学特点、诊断及鉴别诊断。方法:收集4例儿童非典型性骨外尤文肉瘤,对病变组织行HE、免疫组化染色,荧光原位杂交(FISH)检测EWSR1基因断裂,观察其组织学形态、免疫表型及分子遗传学特征。结果:低倍镜下,肿瘤细胞被纤维血管间质分隔呈小叶状或巢片状。其中,2/4例瘤细胞较大,染色质淡染,可见明显核仁;1/4例由小圆蓝细胞、体积较大的胞浆透亮的圆形细胞、核深染的梭形细胞及上皮样细胞构成;1/4例由上皮样细胞组成。CD99、Fli-1阳性比例为4/4,神经内分泌标记呈不同程度阳性,FISH均检测到EWSR1基因断裂。结论:EWSR1基因重排可发生于多种肿瘤,骨外尤文肉瘤组织形态多样,对于不典型病例,其诊断需结合组织形态、免疫组化染色及EWSR1基因检测综合考虑。
Objective:To investigate the histopathological characteristics,diagnosis and differential diagnosis of atypical extraskeletal Ewing sarcoma.Methods:HE staining,immunohistochemical staining and fluorescence in situ hybridization(FISH)gene detection were performed in 4 cases of atypical extraskeletal Ewing sarcoma to observed histological morphology,immune phenotype and characteristics of molecular genetics.Results:The tumors were separated by fibrous vascular connective tissue into small leaf or nested shape.2/4 cases had larger tumor cells with light chromatin staining and obvious nucleoli.1/4 cases had small round blue cells,larger round cells with epithelioid cytoplasm,deeply stained spindle cells,and epithelioid cells.1/4 cases had epithelioid cells completely.The positive ratio of CD99 and Fli-1 were 4/4.Neuroendocrine markers were positive to varying degrees,and FISH detected EWSR1 gene disruption.Conclusion:EWSR1 rerrangement can occur in a variety of tumors,and the morphology of extraskeletal Ewing sarcoma is diverse.For atypical cases,its diagnosis needs to be combined with histological morphology,immunohistochemical staining and EWSR1 gene detection.
作者
刘燕飞
李娟
陈广生
安鲁
张娜
王哲
杨丽
LIU Yanfei;LI Juan;CHEN Guangsheng(Department of Pathology,Xi’an Children’s Hospital,Xi’an 710003)
出处
《陕西医学杂志》
CAS
2020年第3期360-363,369,共5页
Shaanxi Medical Journal
