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目的探讨黏液纤维肉瘤(MFS)临床病理学特征、免疫表型及鉴别诊断要点。方法对4例MFS进行光镜观察和免疫组化标记,并进行文献复习。结果 MFS好发于中老年人,患者多以缓慢增大的皮下无痛性肿块就诊。镜下见肿块呈结节性生长,结节间为纤细而不完整的纤维结缔组织间隔。肿瘤由梭形或星状细胞和富含透明质酸的黏液样间质组成,可见特征性的弧线形血管和假脂肪母细胞。免疫组化显示瘤细胞vimentin(+),例2肿瘤细胞α-SMA和MSA(+),提示有肌纤维母细胞分化。结论根据瘤细胞的丰富程度、细胞核的异型性大小和核分裂的多少可将MFS分为低、中和高度恶性3种。大多数MFS为低度恶性,局部扩大切除即可,部分病例可复发或远处转移。
Objective To investigate the clinicopathological characteristics, immunophenotype and differential diagnosis of mucoid fibrosarcoma (MFS). Methods Four cases of MFS were observed by light microscopy and immunohistochemistry, and the literature review. Results MFS occurs in the elderly, the majority of patients with slowly increasing subcutaneous painless mass treatment. Microscopic tumor showed nodular growth, nodular slender and incomplete fibrous connective tissue interval. The tumor consists of fusiform or stellate cells and myeloid interstitial hyaluronan-rich stroma, showing characteristic arc-shaped blood vessels and pseudofibromocytoma. Immunohistochemistry showed that tumor cells vimentin (+), Example 2 tumor cells α-SMA and MSA (+), suggesting myofibroblast differentiation. Conclusion According to the richness of tumor cells, the heterogeneity of nucleus size and mitosis can be divided into three kinds of low, medium and high malignant MFS. Most of the low-grade MFS malignant, local expansion can be removed, in some cases the recurrence or distant metastasis.