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目的探讨心脏原发性恶性纤维组织细胞瘤的临床病理学特征及诊断、鉴别诊断要点。方法对3例发生在心脏的恶性纤维组织细胞瘤的临床资料、病理形态学、免疫组化结果进行观察,并结合文献探讨。结果 2例患者为女性,1例为男性,年龄20~60岁,平均年龄35岁;均发生在左心房。临床表现无特异性,表现为胸闷、心慌、气促和呼吸困难。镜下瘤组织主要由梭形、卵圆形细胞构成,呈束状、席纹状或不规则排列,并有数量不等的多核巨细胞及少量炎症细胞,坏死多见。免疫组化:3例vimentin(+)、CD68局灶(+),1例SMA局灶(+)。结论心脏原发性恶性纤维组织细胞瘤非常罕见,由于其临床表现无特异性,早期诊断困难,诊断主要依赖于病理组织学及免疫组化检查。
Objective To investigate the clinicopathological characteristics of primary malignant fibrous histiocytoma and its diagnostic and differential diagnosis. Methods Three cases of malignant fibrous histiocytoma in the heart of the clinical data, pathological morphology, immunohistochemistry results were observed, combined with the literature. Results Two patients were female, one was male, aged from 20 to 60 years, with an average age of 35 years; all occurred in the left atrium. Non-specific clinical manifestations, manifested as chest tightness, palpitation, shortness of breath and difficulty breathing. Microscope tumor mainly composed of fusiform, oval cells, were bundles, mylar pattern or irregular arrangement, and a varying number of multinucleated giant cells and a small amount of inflammatory cells, necrosis more common. Immunohistochemistry: 3 cases of vimentin (+), CD68 focal (+), and 1 SMA focal (+). Conclusions Cardiac primary malignant fibrous histiocytoma is rare. Early diagnosis is difficult because of its nonspecific clinical manifestations. The diagnosis mainly depends on histopathology and immunohistochemistry.