韧带样型纤维瘤病CT、MRI表现与病理对照分析  

Computed tomography and magnetic resonance imaging features of desmoid-type fibromatosis:comparison with the pathological findings

作　　者：张忠林[1] 梁长虹[1] 刘于宝[1] 谢淑飞[1] 余元新[1] 王秋实[1] 刘再毅[1] 李景雷[1] 

机构地区：[1]广东省人民医院,广东省医学科学院放射科,广东广州510080

出　　处：《南方医科大学学报》2010年第11期2495-2497,共3页Journal of Southern Medical University

基　　金：广东省科技计划项目(2007B031515008)

摘　　要：目的探讨韧带样型纤维瘤病的CT、MRI表现,提高CT、MRI对该病的认识和诊断水平。方法回顾性分析经临床手术病理证实18例韧带样型纤维瘤病的临床、病理、CT、MRI特征。18例中10例行CT平扫、增强扫描,8例行MRI平扫及增强扫描,分析其CT、MRI表现特征并与病理结果对照。结果腹部外病例发生于头颈部3例,胸背部2例,腹壁、腹股沟9例,腹腔内4例,1例与Gardner综合征伴发。本组中4例发生在腹部手术后1至3年内发病。韧带样型纤维瘤病病理:病变质地较硬。由梭形纤维母细胞和肌纤维母细胞组成。细胞无异型性,核分裂少见或无。局部呈侵袭性生长,有局部复发倾向但不发生远处转移。免疫组化:纤维母细胞和肌纤维母细胞都强表达波形蛋白Vimentin,肌纤维母细胞不同程度表达平滑肌肌动蛋白SMA。CT、MRI表现:良性,但呈现恶性肿瘤生长方式;可推挤周围脏器、血管,或包绕血管;边界多不清,呈浸润性生长,无包膜;坏死或钙化少见;密度、信号特点:均匀。强化方式:12例三期均明显均匀强化;5例不均匀强化。结论韧带样型纤维瘤病具有特征性CT、MRI表现,CT、MRI对韧带样型纤维瘤病的诊断及鉴别诊断具有重要价值。Objective To explore the computed tomography（CT） and magnetic resonance imaging（MRI） features of desmoid-type fibromatosis,and improve the diagnostic accuracy and understanding of the disease.Methods The CT and MRI features of 18 cases of surgically and pathologically confirmed desmoid-type fibromatosis were reviewed retrospectively.Among the patients,10 received CT pre-and post-contrast scanning,and 8 patients had MRI pre-and post-contrast scanning.The CT and MRI features were analyzed in comparison with the pathological findings.Results In the extraabdominal cases,the tumors occurred in the head and neck in 3,in the dorsal part of the chest in 2,in the abdominal wall and groin area in 9,and in the peritoneal cavity in 4;concomitant Gardner syndrome was found in 1 case.In 4 cases the tumor occurred within 1 to 3 years after abdominal surgeries.Pathologically,the lesion was hard and composed of fusiform fibroblasts and myofibroblast.The cells showed no obvious heteromorphism with few karyokinesis,growing invasively and recurrent locally but without distant metastasis.Immunohistochemically,the fibroblasts and myofibroblasts expressed vimentin,and the myofibroblasts were positive for SMA.On CT and MRI,the lesion appeared benign with malignant growth pattern,and caused compression of the adjacent organs and vessels or encasement of the vessels;the border was unclear without encapsulation,and necrosis and calcification was scarce.The density and signal of the tumor were well distributed.Twelve patients displayed obvious enhancement and 5 showed uneven enhancement.Conclusion The CT and MRI features of desmoid-type fibromatosis are characteristic,and CT and MRI are valuable modalities for the diagnosis and differential diagnosis of the tumor.

关 键 词：韧带样型纤维瘤病 病理学 体层摄影术 X线计算机 磁共振成像 

分 类 号：R730.4[医药卫生—肿瘤]