小儿婴儿型纤维肉瘤临床特征、影像学表现及其误诊分析  

The Imaging Manifestations,Clinical Characteristics and Misdiagnosis of Infantile Fibrosarcoma in Children

作　　者：徐守军[1] 杨春兰[2] 曹娟[3] 李鹏[1] 刘龙平 谢丽春 罗娜[1] 裴妮慧 XU Shoujun;YANG Chunlan;CAO Juan(Department of Radiology,Shenzhen Children's Hospital,Shenzhen,Guangdong Province 518026,P.R.China)

机构地区：[1]深圳市儿童医院放射科,518026 [2]深圳市儿童医院血液肿瘤科,518026 [3]深圳市儿童医院病理科,518026 [4]广州医科大学附属第三医院儿科,510530

出　　处：《临床放射学杂志》2024年第4期626-633,共8页Journal of Clinical Radiology

基　　金：广东省高水平临床重点专科项目(编号:SZGSP012);广东省基础与应用基础研究基金项目(编号:2022A1515111121);深圳市医学重点学科建设经费项目(编号:SZXK034);深圳市医疗卫生三名工程资助项目(编号:SZSM202011005);深圳市基础研究专项(自然科学基金)基础研究面上项目(编号:JCYJ20220530155616038)资助。

摘　　要：目的 观察小儿婴儿型纤维肉瘤(IFS)的临床特征及CT表现,并对误诊病例进行分析,以提高该病诊断正确率。方法 回顾性分析2014年2月至2022年1月我院收治的11例IFS患儿资料。结果 11例患儿中,男9例,女2例,年龄4天~3岁8个月,平均(0.52±1.01)岁。术前7例行X线检查,9例行CT或MRI平扫及增强扫描,其中3例同时行CT和MRI平扫及增强扫描。11例患儿中,6例病灶位于四肢,2例位于胸壁,2例位于腹部,1例位于鼻腔。8个病灶呈椭圆形,3个为类圆形。病灶大多体积较大,最大径约2.2~12 cm,平均(6.9±3.0)cm。首次就诊原因主要为局部包块9例、溃疡2例,其他包括腹胀1例、鼻塞伴反复出血1例。X线主要表现为体积较大、稍高密度软组织包块。CT主要表现为稍低密度软组织包块,与邻近肌肉组织边界不清;增强后病灶不均匀强化,其中渐进性强化4例,边缘结节样强化1例。MRI主要表现为T_1WI等或稍低信号,T_2WI稍高或高信号;增强后病灶不均匀明显强化。11例病灶中,内见出血6例,钙化2例,囊变、坏死9例,大血管流空7例,邻近骨受压、变形、局部骨吸收5例。11例患儿中6例误诊为血管源性肿瘤(VT),1例误诊为畸胎瘤(TT),1例诊断为肌源性或神经源性肿瘤,3例未定性诊断。结论 IFS发病率低,特异性不高,常被误诊。全面认识IFS的影像学表现,分析误诊原因,有助于准确诊断。Objective The purpose of this study was to highlight the imaging findings of infantile fibrosarcoma(IFS)on X⁃ray film,CT and MRI,to summarize the clinical characteristics and the factors causing misdiagnosis.Methods We retrospectively analyzed the Imaging characteristics of the patients with histologically confirmed IFS diagnosed in our hospi⁃tals from February 2014 to January 2022.The 11 patients included nine little boys and two little girls,aged from 4 days to 3 years and 8 months,with an average of(0.52±1.01)years.These cases were performed by the following radiological diag⁃nosis.Preoperative X⁃ray examination was performed in seven patients,plain and enhanced CT or MRI scans were performed in nine patients,three were three patients among of them received both plain and enhanced CT and MRI scans simultaneous⁃ly.Results Of the 11 patients,six(6/11)tumors were located in the extremities,two(2/11)were in the chest wall,two(2/11)were in the abdominal and one(1/11)was in nasal cavity.eight lesions were showed in elliptic shaped and three were quasi⁃circular tumor.Most tumors of all cases were large,and that the maximum diameter line of lesions ranged from 22 mm to 120 mm,with an average of(69±30)mm.The main symptom for the first visit was local lump in nine patients,and ulceration in two of them.The other symptoms included abdominal distension in one case and nasal obstruction with repeated bleeding in one case.The main manifestations of X⁃ray were large and soft tissue⁃masses with slightly higher density.The main CT manifestations were soft tissue masses with slightly low density,and the boundary with adjacent muscle tissue was not clear.Enhanced scan showed uneven enhancement of lesions,including progressive enhancement and edge nodular en⁃hancement.The main MRI manifestations were isointensity or slightly hypointense on T1WI,hyperintensity or slightly hyper⁃intense on T2WI.Enhanced scan showed uneven and obvious enhancement of the lesion.Among the 11 lesions,hemorrhage was seen in six,cal

关 键 词：婴儿型纤维肉瘤 远处转移 手术治疗 化疗 计算机体层成像 磁共振成像 误诊分析 

分 类 号：R738.6[医药卫生—肿瘤]