目的 分析腹外型韧带样纤维 瘤病(extra-abdominal desmoid-type fibromatoses)的CT与MRI表现，以提高对 该疾病的影像学诊断水平。方法 回顾性 分析经病理证实的24例腹外型韧带样纤维 瘤病的CT和MRI表现，并与临床资料及组 织病理学对照分析。搜集腹外型韧带样 纤维瘤病共24例，24患者均行影像学检 查，其中20例行MRI检查，增强15例；10 例行CT检查，增强2例，其中6例同时行 CT、MRI检查。对肿瘤的部位、大小、形 态边缘、CT密度、MRI信号及强化方式进 行分析。结果 24例患者中，14例位于四 肢，4例位于胸壁，颈部2例、臀部2例、 蝶窦1例，脊柱旁1例。最小径1.9cm，最 大径11.2cm，呈椭圆形6例，不规则分叶 状18例，边缘清晰5例、模糊19例，14例 (58.3%)包绕血管神经，16例(66.7%) 有 骨膜粘连，3例侵犯骨质(12.5%)。CT显 示，10例病灶均呈稍低密度，密度均匀， 瘤内未见明显钙化、坏死，2例增强检查 呈中等强化。MRI显示，T1WI呈等或稍低 信号，T2WI呈高于肌肉低于脂肪信号，增 强扫描均呈中等或明显不均匀强化，并随 着各期相渐进性强化。其中13例(65%)可 见条索状或小斑片状T1W及T2WI低信号， 增强后未见强化。结论 腹外型韧带样纤 维瘤病是一种少见的中间型软组织肿瘤， CT、MRI表现具有一定特征性，因此对其 术前明确诊断及预后监测具有重要价值。

Objective To study the CT and MRI findings of extra-abdominal desmoidtype fibromatoses and improve its diagnostic accuracy. Methods The data of 24 patients with pathologically proved extra-abdominal desmoid-type fibromatoses were collected. Among all the 24 cases,9 males and 15 females，median age is 46.5 years old.21 cases were primary，3 cases were secondary.10 cases underwent CT examination (2 cases with enhanced),20 cases MRI examination (15 case with enhanced),with both 6 patients. Image findings were evaluated in relation to the following: location, size, morphology, margin, CT density, MRI signal and enchancement pattern. Results Masses were located in limbs (14 cases), chest wall (4 cases), cervical part (2 cases)and buttocks (2 cases), sphenoid sinus (1 cases), paravertebral gutter(1 cases). Lesions were 1.9~11.2cm in size, including oval round masses in 6 cases and lobular masses in 18 cases, the majority (19/24) of lesions demonstrated ill defined circumscribed border. Neurovascular involvement was detected in 14 tumors (58.3%), periosteal adhesion was seen in 16 tumors (66.7%) and bone distruction was detected in 3 cases (12.5%).10 cases performed CT examinations , without necrosis and calcification, were slightly low density, 2 cases demonstrating moderate enhancement. Compared with the muscle signal, the lesions showed slightly low signal on T1WI and showed high and low mixed signal on T2 WI. After contrast enhancement, the lesions showed moderate enhancement. The stripe and patchy non-enhanced structures with low signal intensity on both T1WI and T2W1 were seen in 13 cases (65%). Conclusion Extra-abdominal desmoid-type fibromatoses is a rare intermediate soft tissue tumor. With the CT and MRI findings of lesions specific, CT and MRI examinations have important value in diagnosis and monitor after operation.