肝肾血管平滑肌脂肪瘤临床病理分析  

Clinicopathologic Characteristics of Hepatic and Renal Angiomyolipoma

作　　者：刘汉勇 王晓玫 赖日权[2] 胡维维[3] 成志强 彭全洲 贺黎升 

机构地区：[1]暨南大学第二临床医学院(深圳市人民医院),广东深圳518020 [2]广州军区总医院,广东广州510010 [3]广东省佛山市第一人民医院,广东佛山528010

出　　处：《罕少疾病杂志》2014年第4期8-11,22,共5页Journal of Rare and Uncommon Diseases

摘　　要：目的探讨肝肾血管平滑肌脂肪瘤(AML)临床病理特征、免疫表型、诊断与鉴别诊断。材料与方法对89例AML临床表现、形态学特点及免疫组化进行研究,并复习相关文献。结果 89例AML中,肾脏AML81例,肝脏AML8例,大体常表现为膨胀性生长的肿块;经典组织学改变为呈现不规则厚壁血管,不规则排列的形似平滑肌样的血管周上皮样细胞、脂肪组织三种成分,免疫组化所有病例都不同程度表达HMB-45,Melan-A,SMA,Vimentin等。结论 AML是一种少见的起源于血管周细胞的间叶组织源性肿瘤,多见于女性,组织学上由脂肪组织、平滑肌(梭形或上皮样)、厚壁血管以不同比例混合而成,免疫组化特异性表达HMB45及Melan-A,免疫组化有助于AML诊断与鉴别诊断。Objective To explore the clinicopathologic feature, immunophenotype, diagnosis and differential diagnosis of hepatic and renal angiomyolipoma（AML）. Methods 89 cases of AML were studied with clinical manifestations, morphology and immunohistochemical staining, and its clinical and pathological findings were further analyzed with review the related literature. Results In the 89 cases of AML, 81 were RAML and 8 HAML; AML usually presented as expansile, noninfiltrative masses. Classic AML shows triphasic histology, with tortuous, thick-walled blood vessels, irregularly arranged sheets and bundles of myoid-appearing PEC, and adipose tissue.The immunohistochemistry tests wre positive for HMB45,Melan-A,Vimentin and SMA in all cases. Conclusions HAML and RAML are mesenchymal tumor with female predominance, and derive from perivascular epithelioid cells. They are composed of adipose tissue, smooth muscle and thick-walled, sometimes hyalinized blood vessels in varying proportions,and are expressed HMB45 and Melan-A which are helpful for diagnosis and differentiation of AML.

关 键 词：血管平滑肌脂肪瘤 血管周上皮样细胞分化的肿瘤 肾肿瘤 肝肿瘤 

分 类 号：R322.47[医药卫生—人体解剖和组织胚胎学] R322.61[医药卫生—基础医学]