肾上皮样血管平滑肌脂肪瘤6例的临床病理分析  

Clinical pathologic of renal epithelioid angiomyolipoma in 6 cases

作　　者：封扬[1] 夏燕[1] 吴玲玲[1] 王小双 FENG Yang;XIA Yan;WU Lingling;WANG Xiaoshuang(Department of Pathology,Suzhou Municipal Hospital(Suzhou Hospital Affiliated to Anhui Medical University),Suzhou,Anhui 234000,China)

机构地区：[1]宿州市立医院(安徽医科大学附属宿州医院)病理科,安徽宿州234000

出　　处：《安徽医药》2022年第7期1374-1377,共4页Anhui Medical and Pharmaceutical Journal

摘　　要：目的 探讨肾上皮样血管平滑肌脂肪瘤(EAML)的临床病理特征、免疫组化及鉴别诊断。方法 对安徽医科大学附属宿州医院2017年3月至2020年8月病理科诊断的6例肾脏上皮样血管平滑肌脂肪瘤进行临床病理分析及免疫组化研究,并复习相关文献。结果 6例EAML均为女性,年龄范围35~87岁,平均年龄58.3岁。影像学术前2例考虑为错构瘤,4例为肾脏占位,肾癌待排。显微镜下肿瘤由不同比例的上皮样细胞,血管,平滑肌,脂肪组织和慢性炎细胞构成,上皮样细胞占肿瘤成分的60%以上,部分细胞异型明显,灶区伴坏死。免疫组化梭形细胞抗体黑色素瘤(HMB-45)(5/6)、黑色素A(Melan-A)(5/6),波形蛋白(Vimentin)(6/6),转录因子E3(TFE-3)(1/6),S-100蛋白(S-100)、内皮细胞标志物(CD34)、共同型急性淋巴细胞白血病抗原(CD10)、细胞角蛋白(CKpan)均(-),Ki-67增殖指数1%~10%。结论 EAML是具有恶性潜能的间叶性肿瘤,以增生的上皮样细胞为主,兼具AML的三种成分。临床易误诊,生物学行为有复发及转移倾向,需临床密切随诊。Objective oid angiomyolipoma(EAML).Methods Six case of EAML who were diagnosed by pathology department of Suzhou Hospital Affiliated to Anhui Medical University from March 2017 to August 2020 were analyzed with conventional histopathological and immunohistochemical stains,and the relative literatures were reviewed.Results All 6 patients of EAML were female,with an age range of 35 to 87years,with the average age was 58.3.Before operation,imaging was considered hamartoma in 2 cases,renal space occupying in 4 cases and renal carcinoma need to be excluded.Microscopically,these tumors are composed of varying proportions by epithelioid,vascular,smooth muscle,adipose tissue,and chronic inflammatory cells.Epithelioid cells accounted for more than 60% of the tumor components,some cells with obvious atypia and some with necrosis.Immunohistochemical staining showed that spindle cells were positive for HMB45(5/6),Melan-A(5/6),Vim(6/6),TEF-3(1/6),spindle cells of all cases were not expressed in S-100,CD34,CD10 and CKpan,and Ki-67 proliferation index ranged from 1% to 10%.Conclusions EAML is a mesenchymal neoplasm with malignant potential,dominated by proliferating epithelioid cells,and contains three components of AML.It is easy to be misdiagnosed in clinic,the biological behavior has tendency to recur and metastasize,and needs to be closely followed by clinical.

关 键 词：肾肿瘤 间叶瘤 上皮样血管平滑肌脂肪瘤 临床病理 预后 

分 类 号：R737.11[医药卫生—肿瘤]