摘要目的：探讨骨脂肪硬化性黏液纤维性肿瘤的临床表现、影像学改变和病理特征。方法收集2014年17例脂肪硬化性黏液纤维性肿瘤，分析其临床病理和影像学特征。结果17例脂肪硬化性黏液纤维性肿瘤，年龄26～67岁，平均46岁；男11例，女6例。患者无明显症状或有病变部位疼痛。大部分（13／17）位于股骨粗隆附近。影像学显示为有硬化边缘边界清晰的病灶，MRI示片状异常信号灶，边界清晰光整，T1W为相对均匀的信号，T2W为混杂信号，压脂有明显高信号。该病变呈多种组织学成分的混杂结构，包括纤维和胶原组织、黏液样基质、脂肪瘤样区域、泡沫状组织细胞、Paget病样的钙化和骨化组织。结论脂肪硬化性黏液纤维性肿瘤是一类有独特发生部位和影像学改变的良性纤维骨性病变，其组织学表现也有一定特征性，在骨肿瘤的病理诊断中并不少见，它可能是纤维结构不良等多种良性骨病在微骨折、缺血等因素诱导下形成的退行性终末期改变。

abstractsObjective To explore the clinicopathologic features and imaging diagnosis of 17 cases of liposclerosing myxofibrous tumor ( LSMFT) and to discuss the mechanism of the disease .Methods Cases of LSMFT diagnosed in 2014 were included in this retrospective study .The clinicopathologic features and imaging findings were evaluated .Results There were 17 cases of LSMFT, occurring in 11 men and 6 women with a mean age of 46 years (range, 26-67 years).Patients were asymptomatic or presented with pain localized over the lesions . Most ( 13/17 ) lesions were located in the intertrochanteric region . Radiographs showed well-defined and often extensively sclerotic margin .MRI showed the lesions to be relatively heterogeneous on T1W, and heterogeneous with high signal intensity on T2W with fat suppression. Microscopically , LSMFT was characterized by a complex mixture of histologic elements , including myxofibrous and collagen tissues , lipomatous areas, xanthoma cells, calcification, irregular ossification and pseudo-Paget′s bone. Conclusions LSMFT is a benign fibro-ossesous lesion with unique imaging characteristics and histologic features , occurring preferentially in some locations .It might represent end-stage degenerative changes in other benign bone lesions such as fibrous dysplasia , simple bone cyst and intraosseous lipoma secondary to trauma from forces and ischemic bone injury exerted on the intertrochanteric region of the femur .