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目的探讨肾上皮样血管平滑肌脂肪瘤(EAML)的临床病理特征、诊断、鉴别诊断及预后。方法对11名肾EAML患者的12例肾进行光镜观察和免疫组化染色。结果 11名患者年龄25～52岁,中位年龄36.2岁。其中5人无症状,5人主诉为腰部酸痛,1人表现为发热。肿瘤直径1.3～17cm,平均9.2cm。术前6例疑为肾癌(RCC)。组织学表现肿瘤以增生的上皮样细胞为特征,胞质丰富嗜酸性,细胞核有异型性,核分裂象可见;可伴有出血、坏死。12例肾EAML中小细胞型6例,大细胞型3例,中间型3例,均伴有经典的肾血管平滑肌脂肪瘤(AML)图像。免疫组化:瘤细胞vimentin、SMA和HMB45(+),AE1/AE3、EMA、CD10、CD34、S-100和CD117均(-),1例Ki-67(+)。术后6名患者得到随访(平均24个月),均健在。结论肾EAML的特征以上皮样细胞增生为主,临床和组织学易与RCC或其他肿瘤相混淆,免疫组化有助于鉴别诊断。瘤细胞不典型增生、核分裂、出血和坏死,甚至肾静脉内瘤栓可能提示肿瘤的恶性潜能,但不能作为诊断恶性的依据,诊断恶性必须要有明确的远处转移。
Objective To investigate the clinicopathological characteristics, diagnosis, differential diagnosis and prognosis of renal epithelial angiomyolipoma (EAML). Methods 12 kidneys of 11 patients with renal EAML were examined by light microscopy and immunohistochemistry. Results 11 patients aged 25 to 52 years old, the median age of 36.2 years old. Five of them were asymptomatic, five complained of lumbago and one showed fever. Tumor diameter 1.3 ~ 17cm, an average of 9.2cm. Preoperative 6 cases suspected of renal cell carcinoma (RCC). Histological features of epithelial cells characterized by proliferation of epithelial cells, rich eosinophilic cytoplasm, nuclear atypia, mitotic figures visible; may be associated with bleeding, necrosis. 12 cases of renal EAML small cell type in 6 cases, 3 cases of large cell type, 3 cases of intermediate type, are associated with classic renal angiomyolipoma (AML) images. Immunohistochemistry: The tumor cells vimentin, SMA and HMB45 (+), AE1 / AE3, EMA, CD10, CD34, S-100 and CD117 were all (-) and 1 Ki-67 (+). Six patients were followed up (mean, 24 months), all alive. Conclusion The characteristics of renal EAML mainly epithelial cell proliferation, clinical and histological susceptibility to confusion with RCC or other tumors, immunohistochemistry contribute to the differential diagnosis. Atypical hyperplasia of tumor cells, mitosis, hemorrhage and necrosis, and even renal vein tumor thrombus may suggest the malignant potential of the tumor, but not as a basis for the diagnosis of malignancy, the diagnosis of malignant must have a clear distant metastasis.