脾原发性血管肉瘤5例临床病理分析

Primary angiosarcoma of spleen:a clinicopathologic analysis of 5 cases report and review of literature

目的探讨脾原发性血管肉瘤的临床病理学特征、诊断及鉴别诊断。方法主要从临床表现、大体检查、光镜和免疫组化方面进行观察并随访。结果5例中男性3例,女性2例,年龄在44～60岁,平均52岁。肿瘤最大者20cm×18cm×8cm,最小者5cm×3cm×3cm。免疫组化瘤细胞F8、CD34、CD31和vimentin均(+)。结论脾血管肉瘤罕见,临床缺乏特异性,病理形态多样,诊断困难,免疫组化特异性标记可明确诊断。

Objective To investigate the clinicopathologic features, pathological diagnosis and differential diagnosis of splenic angiosarcoma. Methods 5 cases of splenic angiosarcoma were studied with clinical data analysis, macroscopy, microscopy and immunohistochemical staining. Clinical follow- up was conducted. Results There were 3 males and 2 females with average age of 52 years. Macroscopically, splenic angiosareoma in this study ranged from 5 to 20 cm in diameter. Two cases were multinodular, and three, solitary. Positive rates of immunostaining for CD31, vimentin, CD34 and F8 were 100% respectively. Conclusions Angiosarcoma of the spleen is a rare tumor with pleomorphic histopathological features. It lacks specific manifestations. Immunohistochemical markers can be used for definite diagnosis.