原发性心脏恶性纤维组织细胞瘤病例报道并文献复习

Primary malignant fibrous histiocytoma of heart:a case report and literature review

目的分析原发性心脏恶性纤维组织细胞瘤(MFH)的临床病理特征和免疫组化表型及其鉴别诊断。方法报道1例原发性心脏恶性纤维组织细胞瘤,并结合文献对该类肿瘤的临床病理表现及诊断与鉴别诊断进行讨论。结果本类肿瘤多见于左心房,大体呈结节状,以炎性纤维组织细胞为主,分深浅两层分布,免疫组化肿瘤细胞波形蛋白,CD68,AACT阳性表达;结合蛋白、S-100蛋白、LCA、肌红蛋白、细胞角蛋白、肌动蛋白阴性表达,CD34阳性。结论原发性心脏MFH非常罕见,免疫组化标记有助于肿瘤诊断,早期发现和肿瘤全切除,对治疗和预后有重要意义。

Objective To study the clinicopathologic feature,immunohistochemical phenotypes and differential diagnosis for primary malignant fibrous histocytoma of heart.Methods Reporting one case of primary malignant fibrous histocytoma of heart,clinical and pathological features,the diagnosis and differential diagnosis of the case were discussed combined with relative literature.Results The tumor usually arised from the left atrium.Grossly,the tumor presented tuberasous,main ly composed of inflammatory fibrous histocytes,which showed two layer distribution as deep and superficial.Immuno-histochemically,the tumor cells expressed positivity of vimentin,CD68,AACT;and negativity of desmin,S-100protein,cytokeratin,actin,LCA,myoglobin;positivity of CD34.Conclusion Primary malignant fibrous histiocytoma of heart is extremely rare.Immunohistochemical staining is very helpful to accurate diagnosis,and complete excision of the tumor,thus it has important significance for the treatment and prognosis of MFH.