肾脏上皮样血管平滑肌脂肪瘤临床病理分析并文献复习

目的探讨肾脏上皮样血管平滑肌脂肪瘤（EAML）的临床病理特点及鉴别诊断。方法通过对2例EAML组织病理学观察、免疫组化标记分析，复习文献，讨论其诊断、鉴别诊断、临床生物学行为的可能性评估。结果2例年龄分别为48、29岁，均无肉眼血尿，无结节硬化症；眼观：肿瘤位于肾脏内，肿瘤无包膜，均有出血、坏死；镜检：瘤细胞体积大，呈多边形，胞质丰富，嗜酸性或空泡状，可见核内包涵体，多核巨细胞散在其中。瘤细胞弥漫性实性排列，呈浸润性边缘；2例均未见典型血管平滑肌脂肪瘤（AML）图像。免疫组化染色显示瘤细胞HMB45、Mela—A、CD68阳性，SMA和vimentin散在性阳性，CK、EMA、RCC、CD10均阴性。结论EAML是一种单向性分化的、缺乏AML经典形态的肿瘤，瘤细胞特异性表达HMB45可与其他肾脏肿瘤相鉴别，易复发和出现转移。

Clinicopathological analysis and literature review of renal epithelioid angiomyolipoma

Objective To investigate the clinicopathological features, differential diagnosis of renal epithelioid angiomyolipoma（EAML）. Methods Two cases of EAML were analyzed by light microscopy and immunohistochemistry. The literature on this tumor was reviewed to discuss the histologie features, differential diagnosis and clinical behavior of EAML. Results Two patients were 48 and 29 years old. All cases with no gross hematuria or evidence of tuberous sclerosis, the location of each tumor was in the kidney. Tumors without capsule, with hemorrhage and necrosis. Microscopically, the neoplastic cells were large or huge, exhibited polygonal epithelioid appearance with abundant eosinophilic or clear cytoplasm. Some cells contained nucleoli and hyaline pseudoinclusions. Scattered multinucleated cells were also evident. The neoplastic cells were solid architecture in arrangement. All cases without evidence of AML. Immunohistochemically, the neoplastic ceils were positive for HMB45, Mela - A, CD68 and focally positive for SMA and vimentin, but negative for CK, EMA,RCC and CD10. Conclusions EAML is a monotypic differentiated neoplasm, without evidence of AML, epithelioid cells with more specific HMB45 expression to differential diagnosis with other kidney tumors. Usually, local recurrence and distant or lymph node metastasis occur.