骨脂肪硬化性黏液纤维瘤的影像表现及误诊病例分析

Imaging manifestations and misdiagnosis of liposclerosing myxofibroma fumor

目的:探讨骨脂肪硬化性黏液纤维瘤的影像表现及误诊病例分析,以提高影像对该病的诊断水平。方法:回顾分析2014年1月至2022年11月经手术病理确诊的21例骨脂肪硬化性黏液纤维瘤的影像及病理资料,其中男14例,女7例;年龄21~73岁;病程4~48个月。临床主要表现为患肢疼痛不适。术前均行X线、CT及MR检查。术后对照病理分析各患者的影像资料,总结其影像表现特征,分析误诊病例原因。结果:21例均为原发病灶,位于双侧股骨14例(右侧9例,左侧5例),其中股骨近端(转子间及周围)12例,股骨远端2例,位于胫骨近端2例,肱骨近端2例,桡骨近端1例,髂骨及跟骨各1例。X线及CT显示,所有病灶均呈地图样或者类圆形溶骨性破坏,边缘清晰伴硬化边,骨皮质连续,无骨膜反应及周围软组织肿块,16例病灶内见钙化、骨嵴及不同程度脂肪成分,其密度混杂,5例病灶呈磨玻璃样密度伴少量骨嵴。MR显示,病灶信号欠均匀,T1WI呈等或稍高信号,T2WI呈不均匀高信号,压脂序列病灶内见明显高信号,增强后呈不均匀强化。5例误诊为骨纤维结构不良。结论:骨脂肪硬化性黏液纤维瘤是一种良性纤维骨性病变,多发生于股骨近端,具有较独特的发病部位及影像改变。较典型者影像诊断容易,不典型者易误诊为骨纤维结构不良。

Objective To focus on the imaging features of liposclerosing myxofibrous tumor(LSMFT)and diagnostic challenges to enhance clinical recognition and differential diagnosis accuracy.Methods Retrospective analysis was conducted on the imaging and pathological data from 21 cases diagnosed with LSMFT between January 2014 and November 2022,including 14 males and 7 females(aged from 21 to 73 years;the course of disease ranged from 4 to 48 months).Patient demographics,clinical presentations,and imaging modalities including X-ray,CT,and MRI were reviewed.Pathological findings were correlated with imaging features to delineate diagnostic criteria and identify causes of misdiagnosis.Results All 21 patients represented primary lesions,located in 14 bilateral femurs[9 on the right and 5 on the left;12 proximal femurs(intertrochanteric and peripheral)and 2 distal femurs],2 proximal tibia,2 proximal humerus,1 proximal radius,and 1 ilium and 1 calcaneus.On X-ray and CT,all lesions showed cartographical or quasi-circular osteolytic destruction with distinct and sclerotic margins,continuous bone cortex,absence of periosteal reaction and surrounding soft tissue mass.Calcification,bone ridge and varying degrees of fat components were observed in 16 lesions,displaying mixed density;5 lesions showed ground-glass density with minimal bone ridge.On MRI,the signal intensity of the lesion was heterogeneous.T1-weighted imaging showed iso-to slightly high signal intensity,while T2-weighted imaging demonstrated unevenly high signal intensity.Fat-suppressed sequences depicted significantly elevated signal intensity within lesions,with post-contrast enhancement showing uneven patterns.5 Cases were initially misdiagnosed as fibrous dysplasia.Conclusion LSMFT is an uncommon benign bone tumor,typically localized in the proximal femur but occasionally found in other skeletal sites.Understanding its distinct imaging characteristics is crucial for accurate diagnosis.Typical cases exhibit identifiable imaging patterns,whereas atypical presentations may lead to misdiagnosis as fibrous dysplasia.