血管周上皮样细胞分化肿瘤3例并文献复习  

Case report of perivasucular epithelioid cell tum or and lterature review

作　　者：陈玉芳[1] 周林艳[1] 万美珍[1] 岳灵萍[1] 李紫燕[1] 汪美华[1] 

机构地区：[1]苏州大学附属常州肿瘤医院病理科,江苏常州213000

出　　处：《现代肿瘤医学》2015年第6期853-856,共4页Journal of Modern Oncology

摘　　要：目的:探讨具有血管周上皮样细胞分化的肿瘤(perivascular epithelioid cell tumor,PEComa)的临床病理学特征、免疫表型、诊断及鉴别诊断。方法:应用HE染色及免疫组织化学方法,对3例PEComa进行组织学形态观察、免疫组化标记及文献复习。结果:上皮样肿瘤细胞多呈巢状和片状排列,细胞体积大,多边形或梭形,胞浆丰富,嗜酸性颗粒状或空泡状,呈蜘蛛样结构,细胞核大,但核浆比小。可见核仁,核分裂相少见。特征性的病理表现为肿瘤细胞围绕扩张的血管呈放射状或袖套状排列。免疫表型肿瘤细胞HMB45、Melan A、SMA阳性。结论:具有血管周上皮样细胞分化的肿瘤(PEComa)是一种具有恶性潜能的间叶源性肿瘤,根据其特有的病理特征、结合病史和临床表现、免疫组化及分子基因的检测,可以作出诊断和鉴别诊断,判断其生物学行为和预后需长期密切随访。Objective：To study the clinicopathologic features,immunophenotype and diagnosis of perivascular epithelioid cell tumor（ PEComa）.Methods：Histopathological examination and immunohistochemical assay were used to observe the characteristics of 3 cases of PEComas and relevant litertures were reviewed.Results：Microscopically,epithelioid tumor cells arranged in nests and cluster,The large cell were polygonal and spindle,with abundant cytoplasms that varied from eosinophilic and grannlar to clear.The nuclei were large with small nucleoli,mitosis were rarely present.The typical histological appearance of PEComas showed the tumor cells often arranged around vascular spaces.Immunohistochemical study revealed that all tumor cells were significantly expressed HMB45,SMA,Melan-A.Conclusion：PEComas can be diagnosed according to unigue histological morphological features immunohistochemical and molecular genetic detection.Follow-up was needed to judge prognosis and biological behavior of PEComa.

关 键 词：PECOMA 免疫组织化学 肝脏肿瘤 肾脏肿瘤 肺脏肿瘤 

分 类 号：R730.2[医药卫生—肿瘤]