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腹外型韧带样型纤维瘤病的临床、病理及影像学表现
作者:郑红伟  祁佩红  薛鹏  陈勇  漆剑频  胡道予 
单位:1. 郑州人民医院放射科  河南 郑州 450053 
2.
 华中科技大学同济医学院附属同济医院放射科  湖北 武汉 430030 
关键词:纤维瘤病 腹部/病理学 纤维瘤病 腹部/放射摄影术 体层摄影术 X线计算机 磁共振成像 回顾性研究 
DOI:R730.262;R730.2
出版年,卷(期):页码:2013,28(4):395-399
摘要:
目的 探讨腹外型韧带样型纤维瘤的影像学表现。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。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例轻度欠均匀强化,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 腹部外型韧带样型纤维瘤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 examine the imaging characteristics of extra-abdominal desmoid-type fibromatosis. Methods The clinical data and imaging findings of 15 patients with extra-abdominal demoid-type fibromatosis confirmed by pathology were retrospectively analyzed, among whom 5 cases were examined by plain CT scan and 4 by contrast-enhanced CT scan; 11 cases were examined by plain MRI scan and 10 by contrast-enhanced MRI. Results Thirteen lesions were located in limbs, chest wall,neck or buttocks; 2 lesions located in bone. Thirteen lesions displayed as ovate or lobulated soft tissue mass,2 lesions displayed as irregular patching. Ten lesions had obscure boundary,5 lesions had clear boundary. Mass tended to be large (average diameter 9.7 cm). On CT scan,5 lesions were of low density compared with muscle,only 1 lesion had necrosis. On T1WI,11 lesions were 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 had linear and curvilinear areas of low signal, only 1 lesion had necrosis; 11 lesions demonstrated obviously uneven contrast-enhancement on MRI,3 lesions demonstrated slightly uneven enhancement and 1 lesion demonstrated obviously uneven enhancement on CT,2 of them gradually enhanced in venous phase. The tumor cells were spindle-shaped in light microscope without obviously heteromorphism and nuclear atypia was rare. The tumor presented invasive growth, infiltrating striated muscle and fatty tissue; tumor had a variable amount of intermingled collagen fibers with hyaline degeneration and mucinous degeneration. Conclusion The imaging of extra-abdominal desmoid-type fibromatosis has some characteristics on CT and MRI scan,MRI has an advantage for judgement of the extend of surgical resection.
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