子宫Ewing肉瘤6例临床病理特征分析及文献复习  

Uterine Ewing sarcoma:a clinicopathological analysis of six cases and review of literature

作　　者：张秋颖 张荣君[1] 黄海建 ZHANG Qiu-ying;ZHANG Rong-jun;HUANG Hai-jian(Department of Pathology,Jinjiang Municipal Hospital/Shanghai Sixth People’s Hospital Fujian Division,Jinjiang 362216,China;Department of Pathology,Fuzhou University Affiliated Provincial Hospital,Fuzhou 350001,China)

机构地区：[1]晋江市医院(上海市第六人民医院福建医院)病理科,福建泉州362216 [2]福州大学附属省立医院病理科,福州350001

出　　处：《诊断病理学杂志》2025年第2期133-137,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨子宫Ewing肉瘤(ES)的临床病理学特征,并系统性复习文献。方法收集、分析6例子宫ES的病理学特征,随访治疗、预后并复习文献。结果年龄14~67岁(中位52岁,平均43.5岁)。肿瘤最大径2.5~13 cm(平均7.6 cm,中位6.5 cm)。病理学:未分化小圆细胞肿瘤,弥漫生长,细胞胞质少,核染色质均匀、细致,见小核仁,病理性核分裂象易见,部分呈巢团样排列,可见Homer-Wright菊形团结构,符合经典型Ewing肉瘤(4/6),另两例肿瘤细胞呈上皮样、短梭形,胞质透明、细胞核空泡状,核仁明显,核分裂象易见,并见灶性坏死,间质见厚壁小血管,符合非经典型Ewing肉瘤(2/6)。免疫组化:肿瘤细胞呈阳性表达CD99(6/6,膜)、Fli-1(6/6)、NKX2.2(6/6)、SMARCA4(6/6),Ki-67增殖指数30-80%。基因检测:FLI-1-EWSR1基因融合(4/4),EWSR1基因分离阳性(2/2)。结论子宫ES恶性度高,侵袭性强,进展迅速,临床与影像表现不典型,需与子宫其他肿瘤鉴别,确诊依赖于临床病理学、免疫表型及基因检测。由于子宫ES罕见,治疗上暂未制定标准治疗方案。Objective To investigate the clinicopathological features of uterine Ewing sarcoma(ES)and to systematically review the literature.Methods The pathological data,follow-up,treatment and prognosis of 6 cases of uterine ES were collected and analyzed with review of the literature.Results The age was 14~67 years old(median 52 years and average 43.5 years).The maximum diameter of the tumor was 2.5~13 cm(average 7.6 cm,median 6.5 cm).Pathologically,it was a undifferentiated small round cell tumor with diffuse growth pattern;tumor cells had less cytoplasm,with uniform size of nuclei and detailed chromatin;small nucleoli were noted,and pathological mitotic figures were frequently found.Some tumor cells were in nest-like arrangement,and Homer-Wright rosettes were seen,consistent with the classic Ewing sarcoma(4/6).In other two cases,tumor cells were epithelioid,or short spindle-shaped,with transparent cytoplasm,vacuole-like nuclei and prominent nucleoli;mitotic figures were frequently noted,with focal necrosis and interstitial thick-walled small blood vessels,which were consistent with non-classical Ewing sarcoma(2/6).Immunohistochemically,tumor cells were positive for CD99(6/6,membrane),Fli-1(6/6),NKX2.2(6/6),SMARCA4(6/6),and Ki67 proliferation index was 30-80%.Genetic testing showed FLI-1-EWSR1 fusion(4/4)and EWSR1 broken-apart(2/2).Conclusion The diagnosis of uterine ES depends on clinical pathology,immunophenotyping and genetic testing,and it needs to be distinguished from other uterine tumors.Due to the rarity of uterine ES,no standard treatment plan has been formulated.

关 键 词：子宫 EWING肉瘤 临床病理特征 免疫组化 基因检测 

分 类 号：R737.33[医药卫生—肿瘤]