婴儿型纤维肉瘤的临床及影像学特征分析  

Clinical and imaging characteristics of infantile fibrosarcoma

作　　者：魏珂 高芷欣 刘龙平 曹卫国[1] WEI Ke;GAO Zhixin;LIU Longping;CAO Weiguo(Department of Radiology,Shenzhen Children's Hospital,Shenzhen,Guangdong Province 518038,China;China Medical University,Shenyang 110122,China)

机构地区：[1]深圳市儿童医院放射科,广东深圳518038 [2]中国医科大学,辽宁沈阳110122

出　　处：《实用放射学杂志》2023年第8期1322-1326,共5页Journal of Practical Radiology

基　　金：深圳市医疗卫生三名工程项目(SZSM202011005)。

摘　　要：目的探讨婴儿型纤维肉瘤(IFS)患儿的临床及影像学特征。方法回顾性分析经病理证实的11例IFS患儿的临床和影像学资料,包括发病年龄、性别、发病部位、疼痛和皮肤改变,影像学征象包括密度/信号、大小、边缘、囊变坏死、出血、钙化、强化、瘤内血管、周围骨质和淋巴结,总结分析IFS主要临床表现和好发部位,总结IFS影像特征。结果11例患儿中,生后即发病占45.4%,1岁以下占81.8%(9/11)。四肢6例,鼻腔内1例,腹膜后2例,胸背部2例。CT和MR检查表现为不均匀密度/信号,CT为软组织密度,T1WI为等/稍低混杂信号,T2WI为混杂高信号,边界不清,囊变坏死(9/10)和瘤内出血(6/10)发生率高,可侵犯周围骨质,多数病灶呈明显不均匀强化,可渐进性或开环状强化,瘤内可见迂曲增粗的血管影,少数病例见不定形钙化和动静脉瘘,常不伴周围淋巴结肿大。结论婴儿型纤维肉瘤主要见于1岁以下,尤其是生后即发现的四肢巨大软组织肿块,囊变坏死和出血发生率高,增强表现为明显不均匀强化。Objective To investigate the clinical and imaging features of infantile fibrosarcoma(IFS).Methods Theclinical and imaging data of 1l patients with IFS confirmed by pathological were analyzed retrospectively,including age,sex,location of onset,pain,and skin changes.Imaging signs included density/signal,sizes,margin,cystic necrosis,bleeding,calcification,enhancement,intra-tumoral vessels,peripheral bone,and lymph nodes.The clinical manifestations,predilection sites,and imaging characteristics of IFS were summarized and analyzed.Results Of the 11 patients,45.4%of the patients were found immediately after birth,and 81.8%were under 1 year old(9/11).There were 6 cases in the extremities,l case in the nasal cavity,2 cases in the retroperitoneum,and 2 cases on the chest and back.CT and MR showed heterogeneous density/signal,soft tissue density on CT,equal/slightly lower mixed signal on T,WI,mixed hyper-signal on T,WI,ill-defined boundary,high incidence of cystic necrosis(9/1o)and intra-tumoral hemorrhage(6/10),may invade surrounding bone.Most lesions were obvious heterogeneous enhancement and showed progressive or open ring enhancement,being accompanied by tortuous and thickened vessels visible within the tumor.In a few cases,amorphous calcification and arteriovenous fistula were found.The peripheral lymph nodes of the lesion were usually not swollen.Conclusion Infantile fibrosarcoma mainly occurs in infancy under 1 year of age,especially at birth,which with heterogeneous density or signal,cystic necrosis and bleeding,and obvious heterogeneous enhancement.

关 键 词：儿童 婴儿型纤维肉瘤 计算机体层成像 磁共振成像 

分 类 号：R738.6[医药卫生—肿瘤] R814.42[医药卫生—临床医学] R445.2