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目的认识低度恶性纤维粘液样肉瘤临床特点和病理诊断及鉴别诊断。方法对１７例低度恶性纤维粘液样肉瘤进行临床、病理及免疫组化分析，并随访观察。结果男１０例，女７例，年龄３～７２岁（平均４３．２岁）。病变部位：下肢１０例（其中大腿９例），背部２例，肘部、胸部、颈部、腹股沟和腹膜后各１例。肿瘤直径２．１～１３．５ｃｍ不等，平均７．６ｃｍ。光镜下：瘤细胞小，梭形或星形，核深染有轻度异形，核分裂不常见。组织学特征为：轻至中度丰富细胞的纤维粘液样区和呈漩涡状排列的梭形细胞区在同一肿瘤中交替出现。免疫组化染色，肿瘤细胞波形蛋白阳性。７例有随访结果。结论低度恶性纤维粘液样肉瘤是一种独立存在、进展缓慢的软组织肉瘤
Objective To understand the clinical features, pathological diagnosis and differential diagnosis of low-grade fibrous myxomatoid sarcoma. Methods Clinical, pathological and immunohistochemical analysis of 17 cases of low-grade fibromyosarcoma-like sarcoma were performed and followed up. The results were 10 males and 7 females, aged 3 to 72 years (average 43.2 years). Lesions: 10 cases of lower extremity (including 9 cases of thigh), 2 cases of back, 1 case of elbow, chest, neck, groin, and retroperitoneum. The tumors ranged in diameter from 2.1 to 13.5 cm, with an average of 7.6 cm. Under the light microscope, the tumor cells are small, spindle-shaped or star-shaped, and the nuclear dark staining is slightly abnormal, and the mitosis is not common. The histological features were: fibrous mucous-like areas with light to moderately abundant cells and spindle cells arranged in a whirlpool alternated in the same tumor. Immunohistochemical staining showed that the tumor cells were positive for vimentin. Seven patients had follow-up results. Conclusion Low-grade fibrous myxomatoid sarcoma is an independent and slowly progressing soft tissue sarcoma