上皮样血管平滑肌脂肪瘤16例临床病理特征及分析

Clinicopathologic features of epithelioid angiomyolipomas: an analysis of 16 cases

目的探讨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,提高对本病的认识。方法对16例EAML进行临床特征、病理形态学特点及免疫组化等进行回顾性分析。结果 16例EAML病例中,男性4例,女性12例,平均发病年龄34岁。影像学提示为错构瘤者6例,其余10例均提示为恶性病变。组织学表现以上皮样肿瘤细胞为主,1例见肾门静脉和下腔静脉瘤栓,4例伴出血及囊性变。免疫组化:所有病例瘤细胞均弥漫表达HMB45、SMA和vimentin,不表达EMA、广谱CK。16例EAML患者中14例有完整随访资料,随访时间14~75个月,1例复发,其余患者均未见远处转移。结论 EAML是一种非常少见的具有潜在恶性的间叶源性肿瘤,临床易误诊为恶性,病理检查特别是免疫组化对明确诊断有重要意义,本瘤有复发和转移倾向,术后需密切随访。

Purpose To discuss the clinicopathologic features,diagnosis,differential diagnosis and prognosis of epithelioid angiomyolipoma（ EAML） and to improve understanding of EAML. Methods Retrospective analyses of the clinical features,pathological morphology and immunohistochemistry were conducted in 16 cases of epithelioid angiomyolipoma. Results Of the sixteen cases,there were 4 males and 12 females; the average age was 34 years. There were 6 cases that the imaging showed hamartoma,while the other ten were considered as malignant lesions. Histologically,the tumors were predominantly composed of epithelioid cells,and one case with a tumor thrombus extended into the renal vein and IVC,four cases with hemorrhage and cystic degeneration. Immunohistochemically,the epithelioid cells all were strongly positive for HMB45,SMA and vimentin,14 were positive for Melan A,while EMA and CK protein were negative.There were 14 cases with complete follow-up data for 14-75 months after operation; one case was found recurrence,while the other cases were without metastasis. Conclusions EAML is a rare mesenchymal tumor with malignant potential,clinicians would be easily misdiagnosed as malignant tumors. Identified diagnosis depends on pathological examination,especially immunohistochemistry. Since EAML has a possibility of recurrence and metastasis,a close follow-up would be beneficial after surgery.