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目的 探讨心原发性恶性纤维组织细胞瘤 (MFH)的病理组织学形态、诊断及鉴别诊断。方法 复习 2例原发于心MFH的相关临床资料 ,并对其进行组织学、免疫组化染色及电镜观察。结果 2例MFH无特异性临床症状与体征。肿瘤细胞常排列呈席纹状 ,形态多样 ,主要由梭形的纤维母细胞样细胞和圆形、卵圆形组织细胞样细胞组成 ,并有不等量的多形性巨细胞及炎细胞。瘤细胞vimentin、CD6 8、Lysozyme和α AT(+)。电镜下瘤细胞呈梭形 ,胞质内有丰富的粗面内质网、溶酶体、中间型微丝和少量脂质。结论 原发于心的MFH极为罕见 ,是一种起源于原始间叶细胞的高度恶性肉瘤 ,诊断依赖于病理组织学、免疫组化染色和电镜检查
Objective To investigate the histopathology, diagnosis and differential diagnosis of primary malignant fibrous histiocytoma (MFH). Methods The clinical data of 2 cases with primary MFH were retrospectively reviewed, and histological, immunohistochemical and electron microscopic observations were performed. Results 2 cases of MFH no specific clinical symptoms and signs. Tumor cells often arranged in a striated pattern, the shape is diverse, mainly by the fusiform fibroblast-like cells and round, oval-shaped tissue-like cells, and there are different amounts of polymorphous giant cells and inflammatory cells. Tumor cells vimentin, CD6 8, Lysozyme and α AT (+). Under the electron microscope, the tumor cells were spindle-shaped, with abundant rough endoplasmic reticulum, lysosomes, intermediate microfilaments and a few lipids in the cytoplasm. Conclusions Primary MFH in the heart is extremely rare and is a highly malignant sarcoma that originates from primary mesenchyme. The diagnosis is dependent on histopathology, immunohistochemical staining and electron microscopy