心脏原发性恶性纤维组织细胞瘤3例临床病理观察  

Cardiac primary malignant fibrous histiocytoma:a clinicopathological study of three cases

作　　者：何霞[1] 季军[1] 令文萍[1] 陈小玲[1] 

机构地区：[1]深圳市孙逸仙心血管医院病理科,广东深圳518020

出　　处：《诊断病理学杂志》2015年第4期197-199,211,共4页Chinese Journal of Diagnostic Pathology

摘　　要：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（＋）,l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 study the cfinicopathological features, diagnosis and differential diagnosis of cardiac primary malignant fibrous histiocytoma （MFH）. Methods The clinical characteristics, pathomorphology and immunohistochemistry were observed in three cases of cardiac primary malignant fibrous histiocytoma, with review of related literature. Results Two cases were female and one case was male. The youngest was 20 years and the oldest was 60 years, with an average age of 35 years. The clinical manifestation of the tumor was non-specific. The main symptoms were chest distress, palpitation, anhelation and dyspnea. The tumor was composed of fusiform and orbicular-ovate cells. The predominant pattern was bunchy, storiform and irregular. Disparate quantity of muhinucleated giant cells and a small quantity of inflammatory cells were present. Immunohistochemically, tumor cells were diffusely positive for vimentin （3 cases）, locally positive for CI）68 （3 cases）, and locally positive for SMA （ 1 case）. Conclusion Cardiac primary malignant fibrous histiocytoma is rare. Early diagnosis is difficult due to its non-specific clinical manifestation. The diagnosis of cardiac MFH depends on histopathology and immunohistochemistry.

关 键 词：心脏肿瘤 恶性纤维组织细胞瘤 临床病理 

分 类 号：R732.1[医药卫生—肿瘤]