骨脂肪硬化性黏液纤维性肿瘤的临床诊疗分析

目的探讨脂肪硬化性黏液纤维性肿瘤(LSMFT)的临床诊疗特点。方法收集2014年11月至2019年1月就诊于上海交通大学附属第六人民医院骨科的72例LSMFT患者的临床诊疗资料,分析其临床诊疗特点及影像和病理学特征。结果72例LSMFT患者,无明显性别差异,平均年龄49.2岁;30~59岁患者占77.8%,且50~59岁患者占比最多;发病部位以股骨最常见(84.7%),且以股骨转子间周围为著。X线和CT显示病灶主要呈地图样变化,病灶的边界均较清晰并伴有硬化带,但硬化带的厚度和分布不一。MRIT1W主要为相对均匀的中等或低信号,局部见高信号区,T1W增强病灶区域信号强化不明显或部分区域轻度强化,T2W压脂病灶主要为高信号,混杂少量低信号区域。病理学上,病灶呈多种组织混杂,可见大片纤维成分、黏液样基质、脂肪瘤样区域、泡沫状组织细胞区域以及骨化和钙化组织。11例(15.3%)行活检辅助诊断,4例伴有股骨颈病理性骨折者行人工髋关节置换。结论LSMFT是一类具有独特的影像学和组织学特征的临床病理学疾病。本研究结果显示LSMFT患者的年龄较文献报道更大。临床上应对该疾病给予足够的重视,把握活检和治疗指征。

Diagnosis and treatment of liposclerosing myxofibrous tumor:a single-center report of 72 cases

Objective To investigate the characteristics of clinical diagnosis and treatment of liposclerosing myxofibrous tumor. Methods The clinical data of 72 patients who were admitted to the Department of Orthopedics , Sixth People's Hospital Affiliated to Shanghai Jiao Tong University, diagnosed as liposclerosing myxofibrous tumor, were collected from November 2014 to January 2019. The clinical characteristics and radiological and pathological features were analyzed. Results There was no significant gender propensity;the average age was 49.2 years, and the age group of 30 to 59 years accounted for 77.8% of the whole, and the patients aged 50-59 years accounted for the most part;the most common site was femur (84.7%), especially the intertrochanteric region. X-ray and CT showed that the lesions mainly were a map-like change. The boundaries of almost all lesions were clear and accompanied by sclerotic border, but the width and distributio n of the sclerotic region were variable. MRI T1W showed mainly a relatively uniform medium or low signal and a locally high signal. On T1W contrast, signal enhancement was not obvious or only a few areas were mildly enhanced. T2W SPAIR showed mainly a high signal and a small low signal area. Pathologically, it was characterized by combination of several histological elements including lipoma, fibroxanthoma, myxoma, myxofibroma , fibrous tissue. Eleven patients (15.3%) underwent biopsies and 4 patients with pathological fracture in femoral neck underwent artificial hip replacements. Conclusions Liposclerosing myxofibrous tumor is a clinical pathological disease with unique radiological and histological features. The age distribution of the patients is older than that reported in the literature. Much more attention should be paid to this disease to better grasp its biopsy and treatment indications.