儿童骨肌原始神经外胚层肿瘤的CT和MRI表现

发布时间：2018-06-24 22:00

本文选题：原始神经外胚层肿瘤 + 儿童　；参考：《放射学实践》2015年12期

【摘要】：目的:探讨儿童骨肌来源外周性原始神经外胚层肿瘤(pPNETs)的CT和MRI影像学特点。方法:回顾性分析经我院病理证实的14例骨肌来源pPNETs患儿的CT及MRI影像学表现。其中男8例,女6例;年龄21个月~13岁,中位年龄9岁。10例行CT扫描,其中5例行CT增强扫描;6例行MRI检查,其中5例同时行MRI增强扫描。结果:本组14例中骨来源和软组织来源各7例,位于胸壁4例、肩胛骨、下肢、脊柱旁区和头部各2例、骨盆1例。骨来源pPNETs的CT表现为溶骨性为主的骨质破坏伴有周围软组织肿块,直径3~8cm,边界多模糊不清,增强后呈不均匀强化;主要MRI表现为T1WI上受累骨结构内见稍低信号,T2WI上呈高信号,骨皮质低信号不连续,周围可见囊实性肿块、呈明显不均匀强化。软组织来源的pPNETs CT表现为软组织密度肿块,内部可有低密度坏死区、钙化少见,肿块通常较大,直径5~15cm,增强扫描肿瘤内可见片絮状强化或不均匀轻度强化,邻近骨性结构可受累;主要MRI表现为T1WI上骨质破坏区多呈等信号或稍低信号,T2WI上多呈不均匀等信号及高信号,增强扫描呈不均匀明显强化,其内囊变坏死区无明显强化,周围骨性结构受累时呈略长T1、长T2信号。结论:儿童骨肌来源pPNET的影像学表现特异性不强,但在临床诊断儿童骨骼肌肉来源的恶性肿瘤时需要注意与本病进行鉴别诊断,CT和MRI不仅可以显示肿瘤的范围,同时对肿瘤可切除性的判断以及治疗效果的监测也很有帮助。
[Abstract]:Objective: to investigate the CT and MRI features of peripheral primitive neuroectodermal tumors (pPNETs) in children. Methods: Ct and MRI findings of 14 children with bone-derived pPNETs confirmed by pathology in our hospital were retrospectively analyzed. Among them, 8 cases were male and 6 cases were female, the age of 21 months was 13 years old, the median age was 9 years old. 10 cases underwent CT scan, 5 cases underwent CT enhanced scan and 6 cases underwent MRI, and 5 cases underwent MRI enhanced scan at the same time. Results: there were 7 cases of bone origin and 7 cases of soft tissue origin, including 4 cases located on chest wall, 2 cases in scapular bone, lower extremity, 2 cases in paraspinal area and head, 1 case in pelvis. Ct findings of pPNETs from bone origin showed osteolytic bone destruction with surrounding soft tissue masses, diameter of 3 ~ 8 cm, blurring of the boundary and uneven enhancement after enhancement, and high signal intensity on T _ 1WI and hypointensity on T _ 2WI. The low signal intensity of bone cortex was discontinuous, and cystic masses were seen around it, showing obvious heterogeneous enhancement. PPNETs CT findings of soft tissue origin showed soft tissue dense mass with low density necrotic area, rare calcification, usually larger mass with a diameter of 5 ~ 15 cm. The enhancement scan showed flocculent enhancement or uneven slight enhancement in the tumor. The adjacent bony structures may be involved, the main MRI manifestations are that the bone destruction area on T _ 1WI is mostly iso-signal or slightly low signal intensity on T _ 2WI, and the enhancement scan is not even and obvious, but the dead zone of the internal capsule is not obvious enhancement, and there is no obvious enhancement on T _ 1WI, especially on T _ 2WI, and on T _ 2WI, there is no obvious enhancement in the dead zone. The peripheral osseous structure was slightly longer T 1 and long T 2 signal when involved. Conclusion: the imaging features of pPNET derived from skeletal muscle in children are not specific, but in clinical diagnosis of malignant tumors of skeletal and musculoskeletal origin in children, we should pay attention to the differential diagnosis of this disease. CT and MRI can not only show the range of tumors. At the same time, it is helpful to judge the resectability of tumor and monitor the therapeutic effect.
【作者单位】：首都医科大学附属北京儿童医院影像中心;首都医科大学附属北京儿童医院病理科;
【分类号】：R445.2;R738.7;R730.44

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