肾上皮样血管平滑肌脂肪瘤 6例的临床病理分析

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。

Clinical pathologic of renal epithelioid angiomyolipoma in 6 cases

Objective To explore the clinicopathologic features, immunohistochemistry and differential diagnosis of renal epitheli-oid angiomyolipoma (EAML).Methods Six case of EAML who were diagnosed by pathology department of Suzhou Hospital Affiliatedto Anhui Medical University from March 2017 to August 2020 were analyzed with conventional histopathological and immunohisto-chemical 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 casesand 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 forHMB45 (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, dom-inated by proliferating epithelioid cells, and contains three components of AML. It is easy to be misdiagnosed in clinic, the biologicalbehavior has tendency to recur and metastasize, and needs to be closely followed by clinical.