韧带样型纤维瘤病的X线平片、CT、MR表现及病理对照分析

Comparative analysis of X-ray,CT,MR diagnosis and pathology in desmoid-type fibromatoses

目的探讨韧带样型纤维瘤病(desmoid-type fibromatoses,DF)的影像学表现及其影像学诊断价值。方法搜集经手术病理证实的DF患者16例,男7例,女9例。12例行CT检查(增强9例),4例行MRI检查(增强1例,4例均同时行X线平片检查)。回顾分析韧带样纤维瘤病的CT和MRI表现,并与组织病理学进行对照分析。结果 16例患者共检出16个病灶,其中腹部外型11例,腹壁型3例,腹内型2例。CT平扫均表现为均匀略低软组织密度,边界不清;X线平片、CT骨骼病变显示呈膨胀性、溶骨性改变,病灶内有小梁形成,呈肥皂泡样,压迫性骨吸收破坏,常见不规则骨嵴,大多可见无钙化、骨化的软组织肿块。与肌肉信号相比,MRI T1WI上呈等、稍低混杂信号;T2WI呈高、低混杂信号,增强扫描呈中等以上不均匀强化;所有病灶内均可见条索状、片状T1WI及T2WI上均呈低信号改变的区域,CT、MR无强化。结论 DF是一种具有侵袭性的良性肿瘤,X线平片可以观察骨质病变的整体形态,CT检查可以帮助了解骨质的破坏特点;T1WI均呈低信号,T2WI呈高、低混合信号,大部呈不均轻度强化,局部呈渐进性延迟强;T1WI、T2WI示病变中斑点状及条带状低信号,MRI、CT增强扫描不强化是DT的特征性表现。因此,综合多种影像学检查方法对多数韧带性纤维瘤病能够作出正确的诊断。

[Abstract] Objective To investigate the imaging manifestations and diagnostic value of imageology of the effect of des- moid fibromatosis （desmoid-type fibromatoses, DF）. Methods 16 patients with DF confirmed by operation and patholo- gy, which included, 7 males and 9 females, were examined by CT and MRI. 12 cases underwent CT examination （9 cases with enhanced）, 4 cases MRI examination （1 case with enhanced）, and 4 cases X-ray examination. Retrospective analysis of CT and MRI of desmoid fibromatosis was analyzed and compared with pathology. Results 16 patients with 16 lesions were detected and divided into three types, extra-abodmen in 11 cases, abdominal wall in 3 cases and intra-abdomen in 2 cases. CT scans showed that the lesions presented homogeneous low density as soft tissue and boundary is not clear. X-ray plain film and CT showed that the skeletal lesions hadexpansile, osteolytic change, trabecular formation within lesions, soap bubble, oppressed absorption of bone and irregular bone ridge. Most lesions had not calcification andossification. Compared with the muscle signal, the lesions showed slightly low signal on T1WI and showed highand low mixed signal on T2 WI. After contrast enhancement, the lesions showed moderate enhancement. The stripe and patchy non-enhanced struc- tures with low signal intensity on both TIWI and T2 WI were seen. Conclusion Desmoid fibromatosis is a kind of aggres- sive benign tumor. X-ray can observe the integrity of bone lesions, CT examination can help understand the features of destruction of bone; stripe structure in the lesions on T1 WI, T2 WI and no enhancement of the lesions were a feature of this disease; Therefore, multiple imaging modalities can make a correct diagnosis of desmoids fibromatosis in most cases.