[1]刘燕飞,李 娟,陈广生,等.儿童非典型性骨外尤文肉瘤4例临床病理分析[J].陕西医学杂志,2020,49(3):360-363,369.
　LIU Yanfei,LI Juan,CHEN Guangsheng,et al.Clinicopathologic analysis of 4 cases of atypical extraskeletal Ewing sarcoma in children[J].,2020,49(3):360-363,369.
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儿童非典型性骨外尤文肉瘤4例临床病理分析
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《陕西医学杂志》[ISSN:1000-7377/CN:61-1281/TN]

卷:
49
期数:
2020年3期
页码:
360-363,369
栏目:
临床病理
出版日期:
2020-03-05

文章信息/Info

Title:
Clinicopathologic analysis of 4 cases of atypical extraskeletal Ewing sarcoma in children
文章编号:
DOI:〖HT5K〗10.3969/j.issn.10007377.2020.03.025
作者:
刘燕飞1; 李 娟1; 陈广生1; 安 鲁1; 张 娜1; 王 哲2 ; 杨 丽2 △
1.西安市儿童医院病理科(西安 710003); 2.空军军医大学西京医院病理科(西安 710032)
Author(s):
LIU Yanfei; LI Juan; CHEN Guangsheng; et al.
Department of Pathology,Xi'an Children's Hospital(Xi'an 710003)
关键词:
非典型性骨外尤文肉瘤;  病理诊断;  CD99;  Fli-1;  EWSR1基因重排;  儿童
Keywords:
Atypical extraskeletal Ewing sarcoma;  Pathologic diagnosis;  CD99;  Fli-1;  EWSR1 rerrangement;  Children
分类号:
R738.6
文献标志码:
A
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。
Abstract:
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.

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更新日期/Last Update: 2020-03-25