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目的:探讨心脏原发性血管肉瘤的临床病理特点、诊断和鉴别诊断、治疗及预后特点。方法:对9例心脏原发性血管肉瘤进行光镜形态及免疫组化特征观察,并结合文献分析讨论。结果:9例中,男性5例,女性4例,年龄在19~62岁,平均39岁。镜下见肿瘤形态多样,局部呈大小不规则的血窦样结构,互相连通,腔内壁衬覆肿瘤性内皮细胞,细胞异型性明显,免疫组化:CD31(+),CD34(+),Fli-1(+),ERG(+),Vimentin(+)。结论:心脏原发性血管肉瘤是一种罕见的预后很差的恶性肿瘤,临床表现缺乏特异性,病理形态多样,诊断困难,借助免疫组化特异性标记有助于明确诊断。
Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis, treatment and prognosis of primary angiosarcoma. Methods: 9 cases of primary cardiac angiosarcoma were observed by light microscopy and immunohistochemistry, combined with the literature analysis and discussion. Results: Among the 9 cases, 5 were males and 4 were females, ranging in age from 19 to 62 years with an average of 39 years. Microscopically, the tumors showed various morphological changes and some irregular sinusoid-like structures connected with each other. The inner wall of the cavity was lined with tumorous endothelial cells and the cells were atypical. Immunohistochemistry showed that CD31 (+), CD34 (+), Fli -1 (+), ERG (+), Vimentin (+). Conclusion: Cardiac primary angiosarcoma is a rare malignant tumor with poor prognosis. The clinical manifestations are not specific, pathological and diverse, difficult to diagnose, with the help of immunohistochemical specific markers help to confirm the diagnosis.