上皮样血管平滑肌脂肪瘤临床病理特点,诊断与鉴别诊断.docVIP

6例上皮样血管平滑肌脂肪瘤临床病理特点，诊断与鉴别诊断 张海燕 [摘 要] 目的 探讨上皮样血管平滑肌脂肪瘤(Epithelioid Angiomyolipoma，EAML)HMB45、波形蛋白(Vim) 、和平滑肌特异性抗原(SMA和MSA)弥漫阳性；而上皮细胞膜抗原(EMA)、AE1/AE3呈阴性。结论 上皮样血管平滑肌脂肪瘤是一种具有恶性潜能的间叶源性肿瘤，形态学上常容易误诊，免疫组化对鉴别诊断有重要意义。 [关键词] 上皮样血管平滑肌脂肪瘤；免疫组织化学；诊断与鉴别诊断 [中图分类号] R730.262 Six Cases of Clinicopathologic Analyses and Differential Diagnosis of Epithelioid Angiomyolipoma ZHENG Hai-yan1, HE Miao-xia2* ( 1 Department of Pathology, Nanyang medical school of Henan province, Nanyang, 473000 China 2 Department of Pathology, Changhai Hospital，The Second Millitary University, Shanghai 200433 China) [Abstract] Objective: To explore the clinical, pathologic features and differential diagnosis of EAML. Methods: Five cases of kidney EAML and one case of liver EAML were investigated pathologically and immunohistochemically. Results: Microscopically, the tumor cells of the six cases of EAML were arranged in the shape of nests or sheet, often with perivascular curffing of epithealial cells. The tumor cells had huge volume in round to polygonal shape with abundant eosinophilic cytoplasm. The nucleus were more large and the center of nucleus was obviously emptily bubble shaped. Immunohistochemically, HMB45, MelanA, vimentin, MSA, and SMA in these tumors exhibited positive. While AE1/AE3 and epithelial membrane antigen(EMA)proved negative. Conclusions: The results of this study suggested that the epithelioid angiomyolipoma was a mild but potentially malignant tumor before distant metastases. The immunohistochemistry was of great significance in differentiatial diagnoses. [Key words] Epithelioid angiomyolipoma；mmunohistochemistry； ifferential diagnosis 上皮样血管平滑肌脂肪瘤(Epithelioid Angiomyolipoma, EAML)是近年来逐渐被认识和接受的一种较为少见的肿瘤，国内外仅有少数病例报道(1～2004年WHO泌尿系统和男性生殖器官肿瘤病理学及遗传学分类将该类肿瘤单独列为一类肿瘤(2)。EAML多发生于肾脏，少数发生于肝组织，但由于其组织学变异很大，病理诊断时易与其它一些肿瘤相混淆。本文收集5例肾脏和1例肝脏上皮样血管平滑肌脂肪瘤，分析其临床表现、病理变化及免疫组化特点，旨在探讨其临床病理特点，提供病理诊断及鉴别诊断的依据。 1　材料和方法 1.1　研究对象 病例来自上海长海医院病理科以及南阳高等医学专科学校附属医院病理科2004.01-2008.12年间的外检档案资料。复查其临床资料和相关辅助检查, 并排除其