摘要
目的探讨骨外黏液样软骨肉瘤（extraskeletal myxoid chondrosarcoma,EMC）的临床病理学特征、诊断和鉴别诊断。方法回顾性分析5例EMC的临床病理学特征并复习相关文献。结果 5例患者中男性4例,女性1例,年龄32-62岁,中位年龄52岁。肿瘤位于大腿4例,左胸部1例。镜检：瘤细胞为圆形或卵圆形,形态及大小较为一致,细胞胞质嗜酸性,核小而深染,圆形或卵圆形,呈特征性的细网状、带状或假腺泡状排列,细胞之间为数量不等的黏液样物质。免疫表型：5例肿瘤细胞均强阳性表达vimentin,2例局灶表达S-100,1例表达Syn;不表达CK、p63、EMA、CD34、SMA等。RT-PCR检测显示EMC染色体9号与22号易位。结论 EMC是一种较为罕见的疾病,临床及影像学无特征性,确诊主要依靠病理组织学检查、免疫表型及分子遗传学检测等方法;EMC是一类生长缓慢,但局部复发和转移率高的恶性肿瘤。
Purpose To explore the clinicopathologic characteristics,pathologic diagnosis and differential diagnosis of extraskeletal myxiod chondrosarcoma. Methods The clinical and pathological features were studied with HE and immunohistochemical staining in 5cases of extraskeletal myxoid chondrosarcoma,and the related literatures were also reviewed. Results Extraskeletal myxoid chondrosarcoma was male predominance（ 4 males： 1 female）,and the age ranged from 32 to 62 years with an average of 52 years. Four tumors occurred in thigh and another in chest. Histologically,the neophlasm were composed of round and oval cells. Immunohistochemically,the tumor cells were positive for vimentin in five cases,synaptophysin expressed in one case,S-100 showed focal positivity in two cases,but negative for CK,p63,EMA,CD34,SMA in all cases. Molecular genetics detection shows chromosome exchange between number 9 and 22. Conclusion Extraskeletal myxoid chondrosarcoma is a rare tumor which has no special characteristics in clinical and iconography,and its diagnosis mainly depends on the histopathologic features,immunohistochemistry and molecular genetics. Extraskeletal myxoid chondrosarcoma grows slowly,but have a high tendency for local recurrence and metastases.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2014年第9期1026-1029,共4页
Chinese Journal of Clinical and Experimental Pathology
关键词
骨外黏液样软骨肉瘤
临床病理特征
免疫组织化学
extraskeletal myxoid chondrosarcoma
clinicopathologic characteristics
immunohistochemistry
