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目的对肾脏上皮样血管平滑肌脂肪瘤(epithelioid agiomyolipoma,EAML)的病理诊断、鉴别诊断和预后进行分析。方法2例肾脏 EAML(其中1例为复发病例),复习其临床资料,病理学检查包括常规病理学、免疫组织化学和超微结构,并进行随访。结果光镜下肿瘤均主要由具有多形性和不典型性的上皮样细胞组成,部分区域有明显的血管周上皮样排列;可见出血和坏死;并可见静脉内瘤栓;淋巴结内可见上皮样肿瘤细胞累及。免疫组织化学肿瘤细胞(包括淋巴结内肿瘤)HMB45、平滑肌肌动蛋白(SMA)、神经元特异性烯醇化酶(NSE)和波形蛋白弥漫阳性;S-100、melan-pan 和 CD68散在阳性;而上皮细胞膜抗原(EMA)、AE1/AE3、CK7、CD117、肌肉特异性肌动蛋白(MSA)、结蛋白、白细胞共同抗原(LCA)、CD20、CD45RO、CD30、CD15、嗜铬素(CgA)、突触素(Syn)、bc1-2、雌孕激素受体(ER、PR)和 p53均为阴性。电镜检查可见一些肿瘤细胞内有黑色素小体样的致密颗粒、肌丝、密体,肿瘤细胞外可见不连续的基膜。2例患者手术后10个月状态良好,无肿瘤局部复发和转移征象。结论血管周上皮样排列、寻找经典血管平滑肌脂肪瘤的结构和肿瘤细胞表达HMB45和 SMA 对于诊断和鉴别诊断至关重要。而细胞的不典型性、出血坏死和核分裂象可能只表明肿瘤的恶性潜能;淋巴结受累、肾静脉瘤栓不是恶性的诊断依据;只有远处转移才是恶性的证据。
Objective To analyze the pathological diagnosis, differential diagnosis and prognosis of renal epithelial-derived angiomyolipoma (EAML). Methods Two cases of renal EAML (including 1 case of recurrent disease) were reviewed. The clinical data were reviewed. Pathological examination included routine pathology, immunohistochemistry and ultrastructure, and were followed up. Results All the tumors under light microscopy were mainly composed of epithelial cells with pleomorphic and atypical features. Some regions showed obvious perivascular epithelial-like arrangement. Hemorrhage and necrosis were seen. Intravenous tumor thrombus was seen. In the lymph nodes, epithelial-like Tumor cells involved. HMB45, smooth muscle actin (SMA), neuron-specific enolase (NSE) and vimentin were diffusely positive in immunohistochemical tumor cells (including intra-lymph node tumors); S-100, melan-pan and CD68 were scattered positive; Epithelial membrane antigen (EMA), AE1 / AE3, CK7, CD117, muscle specific actin (MSA), desmin, leukocyte common antigen (LCA), CD20, CD45RO, CD30, CD15, CgA, Syn, bc1-2, estrogen and progesterone receptors (ER, PR) and p53 were negative. Electron microscopy shows some of the tumor cells have dense melanosome-like particles, myofilament, dense body, tumor cells can be seen outside the discontinuous basement membrane. Two patients were in good condition 10 months after surgery, with no signs of local tumor recurrence and metastasis. Conclusion The arrangement of perivascular epithelium, finding the structure of classical angiomyolipoma and the expression of HMB45 and SMA in tumor cells are of great importance in the diagnosis and differential diagnosis. The cell atypia, hemorrhagic necrosis and mitotic figures may only indicate the malignant potential of the tumor; lymph node involvement, renal vein tumor suppository is not a malignant diagnosis; only distant metastasis is malignant evidence.