肝血管平滑肌脂肪瘤临床病理观察

Clinico-pathological Observation of Hepatic Angiomyolipoma

目的:观察肝血管平滑肌脂肪瘤(HAML)的临床病理与免疫组化特点,探讨该病的诊断与鉴别诊断。方法:分析3例手术切除的HAML的临床表现、组织形态学及免疫表型特征,并结合相关文献复习。结果:2例为女性,年龄分别为52岁、26岁,1例为男性,57岁。均无慢性肝炎病史。影像学显示肝脏占位性病变。组织学上表现为血管、平滑肌和脂肪三种成分以不同比例混合组成,均可见髓外造血。经典型2例,上皮样型1例。免疫组化表现为HMB45(+)、MART-1(+)、SMA(+)、CD117(+)、CK(-)、AFP(-)。结论:HAML为罕见间叶性肿瘤,多发于中青年女性,本病确诊须依靠病理形态学改变与免疫组化表达,手术切除预后良好。

Objective：To observe the clinicopathologic and immunohistochemical characteristics and differential diagnosis of hepatic angiomyolipoma（HAML）.Methods：The clinical manifestations,morphology and immune type are analysised in 3 surgically resected tumor specimens,together with reviwing literature.Result：Two cases are females,of 52 and 26 years old,privately the third is male,of 57 yeas old,They had no chronic hepatitis history.While radiographic findings showed occupying lesions in liver.Mixed esitence of blood vessels,smooth muscles and fat could be found and all have extramedullary hemopoiesis.Two cases are classical type,and the other is epithelioid type.Immunohistochemical staining showed that three cases are postive for HMB45、MART-1、SMA and CD117,and negative for CK、AFP.Conclusion：HAML is a rare mesenchymal neoplasm.Definite diagnosis should ke relied on histopathology and immunohistochemistry.The Prognosis is favorable if sugically resected.