肺复合性血管内皮瘤1例报道及文献复习

Composite Haemangioendothelioma of Lung:A Case Report and Review of the Literature

目的:探讨复合性血管瘤(composite hemangioendothelioma,CHE)的临床病理和生物学特点。方法:结合相关文献对1例肺复合性血管内皮瘤伴皮肤和脑转移的临床资料、组织病理特征及免疫组化结果进行分析。结果:镜检见肿瘤组织由良性、中间型及恶性血管多种成分混合组成:肺组织与头皮的真皮及皮下组织的病变相似,为浸润性生长,病变包括上皮样血管内皮瘤(约占50%)、网状型血管内皮瘤(约占15%)、梭形细胞血管瘤(约占20%)、"血管肉瘤样"区域(约占5%)和良性血管病变(约占10%)。颅内病变可见上皮样血管内皮瘤、梭形细胞肿瘤及动静脉畸形区域。免疫组化标记示FⅧRAg(+)、cD34(+)。结论:肺复合性血管内皮瘤属交界性/低度恶性肿瘤,确诊依赖于病理形态学,有复发倾向,少见转移。

Objective：To investigate the clinicopathological and biological characteristics of composite hemangioendothelioma of lung.Methods：The clinical presentation,histopathological observation,and immunohischemical staining were analyzed in one case of composite hemangioendothelioma of lung associated with metastasis in skin and brain,and the related literatures were reviewed.Results： The histological examination showed a lesion was consisted of a mixture of elements between benign and malignant vascular tumors.The changes of lung were similar to those of the dermis and the brain,with a complex mixture of histological patterns resembling epithelioid haemangioendotheliomas （50%）,retiform haemangioendotheliomas （15%）,spindle cell hemangioma （20%） and angiosarcomas （5%）, benign vascular tumors （10%）.Epithelioid haemangioendothelioma,spindle cell hemangioma and arteriovenous malformation were showed in the brain.The vascular endothelial cells were positive for FⅧRAg and CD34.Conclusion：The composite hemangioendothe- lioma of lung belongs to borderline/low-grade malignant group,whose definite diagnosis should be based on the histopathological and immunohistochemical features to exclude other tumors.After removed by surgery the tumor can recur locally.