全身不同部位韧带样型纤维瘤病的CT及MRI表现

CT and MRI features of different parts desmoid-type fibromatoses

目的探讨全身不同部位韧带样型纤维瘤病(desmoid-type fibromatosiss,DF)的CT、MRI表现。方法回顾性分析2011年1月至2016年1月在本院经病理证实的17例DF患者的CT、MRI表现。结果 17例患者中男8例,女9例,年龄20~74岁,平均37岁。共检出病灶18个,其中16例单发,1例多发(2个病灶);病灶位于腹部外8个,腹壁8个,腹内2个;病灶最大长径为11.4cm;18个病灶中12个呈梭形或椭圆形,1个呈长扁形,5个呈不规则或分叶状;2个腹腔内病灶边界清楚,腹部外或者手术切除肿块后复发者5个边界不清、11个边界欠清;8个呈浸润性生长(包括术后复发的3个),10个呈膨胀性生长;2个术后复发病灶与骨质粘连,1个肺尖处病灶周围肋骨呈慢性改变,可见增生骨棘。CT平扫密度等于或稍低于肌肉,多较均匀;MRI平扫T_1WI均呈等或稍低于肌肉信号,多较均匀,T_2WI呈不均匀稍高或较高信号。CT及MRI增强扫描以中等以上渐进性强化为主,强化均匀或不均匀。结论 DF的影像学表现具有一定特征性,有助于术前诊断,并可显示病灶范围及周围结构受累情况。

Objective To investigate the CT and MRI features of different parts desmoid-type fibromatoses （DF）. Method The images of 17 cases with DF proved by pathology were reviewed retrospectively. Result 18 lesions were discovered, among which 16 cases were solitary while 1 cases had two lesions. 8 lesions were extra-abdominal, 8 ones in the abdominal wall and 2 ones in the intra-abdominal. The largest diameter of lesions was 11.4cm. 12 lesions displayed ovoid or elongated shape, 1 one was flat shape and 5 ones were irregular. The boundary of 2 lesions in the intra-abdominal was clear, the boundary of 5 ones including extra-abdominal and postoperative recurrence was unclear, the boundary of 11 ones were less clear. 8 lesions grew aggressively （including 3 postoperative recurrence）, while 10 ones grew expansively. 2 postoperative recurrence lesions adhere to bone, the lung tip ribs around one lesion was chronic change, bone hyperplasia could be seen. On CT, lesions appeared iso-density or slightly hypo-density compared with normal muscles, mostly homogeneous. On MRI, the lesions presented signal intensity similar to or slightly higher than that of muscles on T1WI and most were homogeneous. On T2WI, all were heterogenous with slightly high or high intensity. All lesions exhibited moderate or obvious enhancement after contrast administration on CT or MRI, and most was homogeneous or heterogeneous gradual strengthening. Conclusion DF has some characteristics on images which are valuable for accurate pre-operative diagnosis, and for evaluation of the lesion extent and involvement of adjacent structures.