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骨外黏液样软骨肉瘤病理与鉴别诊断附4例报道并文献复习
引用本文:于鸿,王朝夫,朱雄增.骨外黏液样软骨肉瘤病理与鉴别诊断附4例报道并文献复习[J].临床与实验病理学杂志,2008,24(5).
作者姓名:于鸿  王朝夫  朱雄增
作者单位:1. 江苏省泰州市人民医院,病理科,泰州,225000
2. 复旦大学附属肿瘤医院,病理科/复旦大学上海医学院肿瘤学系,上海,200032
摘    要:目的 探讨骨外黏液样软骨肉瘤(extraskeletal myxoid chondrosarcoma,EMC)的临床病理特点、免疫表型及病理诊断与鉴别诊断要点.方法 收集4例EMC,对其临床、病理组织学及免疫表型进行观察并复习相关文献.结果 4例患者均为成年男性,平均年龄40.2岁,平均病程约30.3个月.主要表现为无痛性或有触痛性软组织肿块,3例发生于下肢,1例发生于胸壁.病理检查:(1)眼观见瘤体最大径平均6.5 cm,切面呈多结节状,有黏液感,界清.(2)镜检见肿瘤呈分叶状生长,瘤细胞排列呈索状、簇状或纤细网状.3例间质富于黏液样基质,1例富于软骨样基质,血管较稀疏.免疫组化:4例均表达Vim(4/4),3例表达S-100蛋白(3/4),2例表达NSE(2/4),2例表达CgA和Syn(2/4),1例表达CKpan(1/4),1例Ki-67阳性率为50%,3例Ki-67阳性率均<5%,4例均不表达SMA和EMA.结论 EMC是一种罕见的软组织肿瘤,其诊断主要依靠发生部位和组织病理学特征,免疫组化标记可帮助诊断和鉴别诊断.

关 键 词:骨外黏液样软骨肉瘤  诊断  鉴别诊断  免疫组织化学

Extraskeletal myxoid chondrosarcoma:a report of 4 cases and review of literature
YU Hong,WANG Chao-fu,ZHU Xiong-zeng.Extraskeletal myxoid chondrosarcoma:a report of 4 cases and review of literature[J].Chinese Journal of Clinical and Experimental Pathology,2008,24(5).
Authors:YU Hong  WANG Chao-fu  ZHU Xiong-zeng
Institution:1Department of Pathology;Tai-zhou People's Hospital;Jiang-su Province;225000;China;2Department of Pathology;Cancer Hospital of Fudan University;Department of Oncology Shanghai Medical College of Fudan University;Shanghai 200032;China
Abstract:Purpose To investigate the clinicopathological feature,immunophenotype of extraskeletal myxoid chondrosarcoma(EMC) as well as the key points to the diagnosis and differential diagnosis of EMC.Methods Clinicohistopathological characteristics and immunophenotype were studied in four EMC cases.The literatures in point were reviewed.Results Clinic materials all the four cases were adulthood male with the average age in 40.2 years old,and the average duration of symptoms for about 30.3 months.The patients primar...
Keywords:extraskeletal myxoid chondrosarcoma  diagnosis  differential diagnosis  immunohistochemistry  
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