肝脏血管平滑肌脂肪瘤临床病理分析

目的 探讨肝脏血管平滑肌脂肪瘤(HAML)的临床病理特征.方法 结合文献对1例HAML的临床资料、免疫组化、超微结构和随访结果进行分析.结果 HAML女性好发,成人多见,常无症状,体检发现:影像学因肿瘤成分构成比和分布不同而异;肿瘤由厚壁畸形血管、成熟脂肪和多形肌样细胞构成,并常见有单核、多核和巨核细胞呈散在巢团状集聚和灶性髓外造血灶;上皮样肌样细胞HMB45、CD117阳性及超微结构见不同成熟时期黑色素小体;治疗以手术为主.结论 HAML主要由血管、脂肪、平滑肌3种成分组成,其中变化最大的是肌样细胞,肌样细胞HMB45标记及电镜检查不同发育时期黑色素小体是诊断HAML的关键.HAML多为良性,首选手术,但有恶性HAML的报道.

A Clinicopathological Analysis of Hepatic Angiomyolipoma

Objective To study the clinicopathological features of hepatic angiomyolipoma(HAML).Methods One case with HAML was studied clinicopathologically,immunohistochemically,ultrastructurally and follow-up combined with literature.Results the most patients were adults and female predominance,and most of the patients had no symptoms.In imaging examinations,it was variety with the tumor component and histostructure.Microscopically,HAMLs showed a varying admixture of thick blood vessel,mature adipose tissues and multimorphological muscle-like cells,with scattered foci of mono-and multi-nucleated cells,and hematopoiesis. Most epithielioid muscle-like cells were positive for HMB45,CD117 in immunohistochemical stainings and premelanosomes in ultrastructural studies.The treatment strategies were surgical operation.Conclusions HAMLs were composed of blood vessels,adipose tissues and smooth muscles.In three components,the most varying was muscle-like cells.HMB45 marker and premelanosomes in ultrastructural observance were key to pathological diagnosis of HAML.Most of HAMLs were benign.The treatment strategies were surgical resection.