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、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 study the clinicopathologic features, diagnosis, differential diagnosis and prognosis of epithelioid angiomylipoma （EAML） of the kidney. Methods Twelve renal EAMLs from eleven patients were analyzed through light microscopy and by immunohistochemical staining. Results The age of the patients ranged from 25 to 52 years （mean 36. 2 years）. Five patients were asymptomatic, five complained with flank pain and one presented with fever. The mean size of EAMLs was 9. 2 cm （range 1.3 to 17 cm）. Six cases were suspected to be renal cell carcinoma （RCC） before surgery. Histologically, the tumors were predominantly composed of epithelioid cells, which were full of abundant eosinophilic cytoplasm. Nuclear pleomorphism and mitosis were seen. Hemorrhage and necrosis appeared. The epithelioid tumor cells were categorized into three types. In our group, six cases were small cell type, three were large cell type, and other three were intermediate type. Twelve cases of EAML accompanied with typical angiomylipoma （AML） of kidney. Immunohistochemically, tumor cells were positive for vimentin, HMB45 and SMA, while AE1/AE3, EMA, CD10, CD34, S-IO0 protein and CD117 were negative, and only one case of EAML was positive for Ki-67. l%llow-up data were available for six patients with a mean of twenty-four months, and they all remained well. Conelluslon Renal EAMLs are characterized by hyperplastic epithelioid cells. EAML of kidney should be differentiated from RCC and other renal tumors in clinicopathologic diagnosis. Immunohistochemistry is of great significance in differential diagnosis. The level of cytologic atypia, mitosis, presence of hemorrhage and necrosis, even tumor emboli in renal vein may indicate the malignant potential of this tumor rather than malignancy. Definitive evidence of malignancy requires demonstration of distant metastasis.
出处
《诊断病理学杂志》
CSCD
2010年第5期321-324,共4页
Chinese Journal of Diagnostic Pathology
