肾恶性上皮样血管平滑肌脂肪瘤临床病理观察  

Clinicopathological features of malignant epithelioid angiomyolipoma in kidney

作　　者：徐丹[1,2] 陈洈伟 孙利[1] 杨书雄[2] 周立新[1] 黄小征[1] 林冬梅[1] 李忠武[1] 

机构地区：[1]北京大学肿瘤医院暨北京市肿瘤防治研究所病理科,恶性肿瘤发病机制及转化研究教育部重点实验室,北京100142 [2]松滋市人民医院病理科,湖北松滋434200

出　　处：《诊断病理学杂志》2016年第12期946-948,共3页Chinese Journal of Diagnostic Pathology

基　　金：首都临床特色应用研究(Z141107002514077);北京市科技新星计划(2010B033)

摘　　要：目的探讨肾恶性上皮样血管平滑肌脂肪瘤（EAML）的临床病理学特征。方法对1例肾高度侵袭性EAML进行组织学及免疫组化分析,并复习相关文献。结果巨检：肿瘤呈多结节状,界限不清,浸润性生长。镜检：瘤细胞主要呈上皮样,只有极少量的梭形细胞,肿瘤中见散在的厚壁血管,未见明显脂肪组织。瘤细胞轻~中度异型,偶见重度异型,病理性核分裂象〉2个/50HPF,可见脉管瘤栓及淋巴结累及。免疫组化：瘤细胞SMA、actin、caldesmon和cathespin k（＋）,HMB45局部（＋）,desmin、CD34、CD117、melan-A、EMA、CK、RCC、ERG、FⅧ、CD31、Syn、Cg A、CD56和melan-A均（-）。结论肾EAML是具有恶性潜能的间叶性肿瘤,高度侵袭性EAML极为少见,其具有特定的形态及免疫组化表型。Objective To investigate the clinicopathological features of malignant epithelioid angiomyolipoma（EAML）in kidney.Methods A case of malignant EAML in kidney was studied with histology,immunohistochemistry and literature review.Results Grossly,the tumor was multinodular with unclear margin and invasive growth pattern.The majority of tumor cells were epitheliod and few of them were spindle type;thick-walled blood vessels were scattered in the tumor.No apparent fat tissue could be observed.Most of the tumor cells were mild to moderate atypia,but severe atypia could be detected occasionally.Immunohistochemical staining showed that the tumor cells were positive for SMA,actin,Caldesmon,Cathespin k,and HMB45,but negative for desmin,CD34,CD117,EMA,CK,RCC,ERG,F Ⅷ,CD31,Syn,Cg A,CD56 and melan-A.Conclusion EAML is a mesenchymal tumor with malignant potential,and high malignant EAML is very rare tumor which has specific histological and immunohistochemical features.

关 键 词：血管平滑肌脂肪瘤 恶性 上皮样 诊断 

分 类 号：R737.11[医药卫生—肿瘤]