儿童韧带样型纤维瘤病的CT与MRI表现

CT and MRI features of desmoid-type fibromatosis in children

目的探讨儿童韧带样型纤维瘤病(DF)的CT、MRI表现,以提高对本病的认识。方法选取经手术及病理证实的23例DF患儿,分析其CT和MRI特征。23例患儿共进行了21次CT及11次MRI检查,其中6例均行CT和MRI检查,CT增强20例,MRI增强9例。结果23例患儿中22例(95.6%)为单发实性肿块,边界多不清晰16例(69.6%),轮廓不光整15例(65.2%),无包膜21例(91.3%),侵袭性生长21例(91.3%)。23例中腹壁外型13例(56.5%),腹壁型8例(34.8%),腹内型(肠系膜来源)2例(8.7%)。肿块最大径范围22~171 mm,平均(65.3±34.7)mm。CT平扫显示肿块呈等密度,密度均匀或不均匀,CT增强扫描表现为轻度强化或延迟扫描呈渐进性强化。MRI表现为T_(1)WI等或低信号,T_(2)WI高信号,内部信号欠均匀。1例腹外型DF T_(1)WI低信号,T_(2)WI低信号,内部信号较均匀。MRI增强扫描显示病灶呈中度不均匀强化伴内部条索状、片状无强化区。结论儿童DF影像学表现有一定特征:肿瘤呈侵袭性生长,CT增强扫描显示肿瘤为渐进性或持续性强化,MRI则更能反映病灶内特征性胶原纤维的低信号。

Objective To investigate the CT and MRI manifestations of desmoid-type fibromatosis(DF)in children.Methods The imaging performance of 23 cases with DF proved by surgery and pathological examination was analyzed retrospectively,and their CT and MRI features were analyzed.Results In all 23 children,22 cases(95.6%)showed single solid mass,the lesion boundary in 16 cases was not clear(69.6%)and 15 cases were irregular in contour(65.2%),21 cases showed no envelope and invasive growth(91.3%),including that 13 cases(56.5%)were external abdominal wall type,8 cases(34.8%)were abdominal wall type,and 2 cases(8.7%)were intra-abdominal type(mesentery).CT examinations showed equal density and uniform density.The maximum diameter of the mass was 22~171 mm(65.3±34.7)mm.Contrast enhanced CT scan showed mild enhancement and gradually delayed enhancement.On MRI,tumors showed isointensity or slightly hypointensity on T_(1)weighted image(T_(1)WI)and with hyperintensity on T_(2)weighted image(T_(2)WI).But,in 1 case,the abdominal DF showed low signal on T_(1)WI and T_(2)WI,and the signal was uniform.Lesions in contrast MRI scanning showed moderate and obviously uneven enhancement,with some non-enhancement areas.Conclusion Pediatric DF has certain characteristics.The tumor shows aggressive growth and persistent enhancement in CT and MRI and hypointensity on T_(2)WI can better revel the histological characteristics of collagenous fiber in the lesions.