摘要
目的探讨儿童腹壁韧带样纤维瘤病(DF)的影像学表现和病理特征,以提高对该病的认识和影像诊断水平。方法回顾性分析本院2011年5月至2020年6月经病理证实的9例儿童腹壁DF的临床和影像表现特征,观察指标主要包括病灶的部位、形态、数目、大小、边界、生长方式(浸润性/膨胀性)、与周围肌肉组织结构关系、密度、信号和强化特征。所有标本均行常规石蜡切片、HE染色和相关免疫组织化学染色检查。结果9例患儿的中位年龄为4岁,男性7例、女性2例;均为单发病灶,病变位于腹壁肌肉,4例突向腹腔生长;病灶呈椭圆形或梭形7例,沿肌间隙蔓延呈不规则形2例。CT平扫呈等或稍低密度,未见明显钙化、坏死或囊变,增强扫描呈渐进性强化;MR平扫T1WI病变呈等/稍低信号,T2WI信号不均匀,呈低/等或等/高信号,增强扫描不均匀强化,1例病灶内见不强化低信号条索影,1例DWI呈明显高信号,ADC与周围肌肉信号相等。手术所见肿瘤质地硬,仅1例可见包膜,其余均和周围肌肉组织分界不清。病理镜下肿瘤由不同比例的梭形细胞和胶原纤维组成,7例结合免疫组化者Vimentin均阳性。结论儿童腹壁DF的临床、病理及影像表现具有一定特征性,不同影像检查方法的应用,对临床术前正确诊断具有重要价值。
Objective To investigate the imaging findings and pathological features of abdominal desmoid fibromatosis(DF)in children,as to improve the understanding and imaging diagnostic level of DF.Methods Imaging findings in 9 patients with abdominal DF verified by clinic and pathology were analyzed retrospectively from May 2011 to June 2020.The main outcome measures included the location,shape,number,size,boundary,growth pattern(infiltration/expansion),relationship with surrounding muscle tissue structure,density,signal and enhancement characteristics.All specimens were examined by paraffin section,HE staining and immunohistochemistry.Results There were two females and seven males with an median age of 4 years.All were single lesions,located in the abdominal wall muscles,and 4 cases protruded into the abdominal cavity.Seven cases were elliptic or spindle-shaped lesions,and 2 cases were irregular spreading along the muscle space.On plain CT scan,tumors showed equal or slightly lower than the density of muscle with no obvious calcification,necrosis or cystic transformation.Enhanced scan showed progressive enhancement.The lesions showed equal or slightly low intensity signal on T1WI,heterogeneous signal(low-equal or high-equal)on T2WI,and inhomogeneous enhancement on contrast enhanced scan.One case found low band signal.One case of DWI showed obvious high signal with ADC intensity signal equal to the muscle.The tumor was hard in texture,and only 1 case had a capsule.The rest were unclear from the surrounding muscle tissue.The tumor was composed of different ratios of spindle cell and collagen fibers under the pathological microscope.Vimentin were positive for seven cases underwent immunohistochemistry.Conclusion The clinical,pathological and imaging manifestations of children with abdominal DF have certain characteristics.The application of different imaging examination methods is of great value for clinical preoperative diagnosis.
作者
李小会
陈桂玲
盛会雪
顾海斌
杨明
高峰
LI Xiaohui;CHEN Guiling;SHENG Huixue;GU Haibin;YANG Ming;GAO Feng(Department of Radiology,Children's Hospital of Nanjing Medical University, Nanjing 210008, China)
出处
《临床肿瘤学杂志》
CAS
北大核心
2020年第12期1125-1128,共4页
Chinese Clinical Oncology
关键词
腹壁韧带样纤维瘤病
计算机体层摄影
磁共振成像
儿童
Abdominal desmoid fibromatosis
Computed tomography
Magnetic resonance imaging
Children
