韧带样纤维瘤病MRI特征表现及其病理基础

目的：探讨韧带样纤维瘤病（DF）的MRI特点，重点分析MRI特征影像及其病理基础，评价MRI在DF诊断中的价值。方法回顾性分析经手术病理证实14例韧带样纤维瘤病的MRI表现（14个病灶均行M RI增强及延迟扫描），与术后病理结果进行比较，并探讨M RI特征影像的病理基础。结果14例均为单发病灶（腹外型7例、腹壁型4例、腹内型3例），其中有2例为术后复发，肿瘤沿着肌肉长轴生长。T1 WI呈等或稍低于肌肉的信号，T2 WI呈高于肌肉信号，增强扫描病灶均呈不均匀强化，以延迟强化为明显，病灶内可见条状或带状 T1 WI及 T2 WI低信号，增强后未见强化。14例病灶内均未见坏死、囊变及瘤周水肿。病理组织学上肿瘤由不同比例的成纤维细胞、纤维组织和胶原纤维束组成，纤维细胞及成纤维细胞密集区域T2 WI信号增高，而肿瘤内胶原纤维区域在 T1 WI、T2 WI均为低信号。肿瘤内见较多血管壁完整的毛细血管，这是病灶延迟强化的病理基础。结论病灶内可见T1 WI、T2 WI均呈带状低信号且增强扫描不强化以及病灶延迟强化是DF的特征性MRI表现。MRI可以很好评估肿瘤侵犯范围及其与周围结构的关系，有助于DF的诊断和鉴别诊断。

MRI manifestation and correlation with pathologic findings in the diagnosis of desmoid-type fibromatosis

Objective To explore the magnetic resonance imaging （MRI） features of desmoid-type fibromatosis （DF） ,to analyze emphatically the correction of M RI characteristic features and pathological basis of DF ,and to evaluate the value of MRI in the diagnosis of DF .Methods The MRI imaging characteristics of 14 patients with DF confirmed pathologically were retrospectively analyzed （all the patients were scaned with contrast enhance ） and compared with findings of pathology .Results 14 cases were single lesion （7 lesions were extre-abdominal ,4 lesions were located in the abdominal wall ,others were intra-abdominal） ,two of which were postoperative recurrence .The tumors developed along the long axis of muscle .The tumors were show isointense or hypointense on T1-weighted and hyperintense on T2-weighted relative to muscle , all lesions were heterogeneous enhancement ,especially on delay-enhancement .There were speckle-shaped and strip-shaped areas of low signal intensity on both T1WI and T2WI within the tumors ,which were not-enhanced .There were no necrosis ,cystic and surrounding edema in the lesions of all the cases .It was revealed by pathology study that the tumor was composed of different proportions of lively proliferative fibroblasts ,fibrocytes and collagenous fiber bundles .Compact fibroblasts and fibrocytes areas showed higher SI on T2WI .There was abundant blood capillary in the tumor ,which was the lesson for pathological foundation of delay-enhancement .Conclusion There were no-enhanced speckle-shaped and stripshaped areas of low signal intensity on both T1WI and T2WI within the tumors ,and the tumor was delay-enhanced ,which were the MRI features of DF .MRI can well evaluate the scope of tumor invasion and its relationship with surrounding structures ,as well as be helpful for diagnosis and differential diagnosis of DF .