低度恶性纤维粘液样肉瘤临床病理研究

Study of clinical pathology in low-grade fibromyxoid sarcoma

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。

Objective To study the clinical features,pathological diagnosis and differential diagnosis of low-grade fibromyxoid sarcoma. Method Clinicopathological and immunohistochemical analysis was carried out on 17 cases of low-grade fibromyxoid sarcoma. Results Male patients were 10 and female 7,whose age ranged from 3 to 72 years.Tumours were located at the lower limb (10 cases,9 in the thigh),the back (2 cases) and the elbow,chest wall,neck,groin and retroperitoneum (1 case each). The maximum dimension of tumours varied from 2.1 to 13.5 cm (mean 7.6 cm).Tumour cells were small,spindle to stellate. There was minimal nuclear pleomorphism and mitoses were uncommon. All tumours were charaterized by the presence of bland spindle cells,arranged in whorls,alternating with a fibrous or myxoid stroma. Immunohistochemical stains demonstrated Vimentin positivity.Follow up was done in 7 cases. Conclusion Low-grade fibromyxoid sarcoma is a distinctive indolent soft-tissue sarcoma.