腹外型韧带样型纤维瘤病的影像学表现及病理分析

Imaging Features and Pathological Analysis of Extra-Abdominaldesmoid-Type Fibromatoses

目的探讨腹外型韧带样型纤维瘤病的影像学表现。方法回顾性分析经病理证实的15例腹外型韧带样型纤维瘤病患者的临床及影像资料。其中MRI检查11例,增强10例;CT检查5例,增强4例。结果 13例位于四肢、胸壁、颈部及臀部软组织内,2例位于骨内。形态上,13例呈类圆形或分叶状肿块,2例呈不规则斑片状影,10例边界不清或部分不清,5例边界尚清。肿块直径较大,平均直径约9.7 cm。CT显示,5例病灶均呈略低于肌肉的低密度灶,其中1例伴囊变、坏死;MRI显示,11例病灶与肌肉比较均呈不均匀等或稍长T1、稍长T2信号,内均伴条片状长T1、短T2信号,其中1例伴囊变、坏死。MRI增强10例呈不均匀明显强化;CT增强4例,3例略不均匀轻度强化,1例不均匀明显强化,2例静脉期呈渐进性强化。病理镜下瘤细胞呈长梭形,无明显异型性,核分裂少见,呈侵袭性生长,浸润周围的横纹肌及脂肪组织,伴胶原纤维增生及程度不等的透明变性、黏液变性。结论腹外型韧带样型纤维瘤病的影像学表现具有一定的特征性,且MRI对手术切除范围的判断具有优势。

Objective To study the imaging characteristics of extra-abdominaldesmoid type fibromatoses. Methods 15 cases proved by pathology were retrospectively analyzed with clinical data and imaging appearance,5 cases plain scan and 4 enhanced, by CT, 11 cases plain scan and 10 enhanced by MRI. Results 13 focuses located in four limb, chest wall, cervical part and buttocks; 2 focuses located in bone. 13 lesions displayed ovate or lobulated soft tissue mass, 2 le- sions displayed irregular patching;10 lesions obscure boundary ,5 lesions clear boundary. And mass tended to be large （av- erage diameter 9.7cm）. On CT ,5 lesion appeared low density Compared with muscle, only 1 lesion with necrosis; On T1WI, 11 lesions were usually heterogeneous with signal intensity similar to or slightly higher than that of muscles and on T2WI they presented prominent heterogeneity with low or high signal intensity, all lesions with Linear and curvilinear areas of low signal, only 1 lesion with necrosis;10 lesions demonstrated obviously uneven enhancement on MRI after contrast, 3 lesions demonstrated slightly uneven enhancement and 1 lesion demonstrated obviously uneven enhancement on CT after eontrast,2of them gradually enhanced in venous phase. The tumor cells was spindle in light microscope without obviously heteromorphism, nuclear atypia was no seen, invasive growth, infiltrated striated muscle and fatty tissue aroundly, with a variable amount of intermingled collagen fibers with hyaline degeneration and mucinous degeneration. Conclusion The imaging of extra-abdominaldesmoid-type fibromatoses has some characteristics, MRI has advantages in respect to the assess- ment of extent of surgical resection.