儿童恶性外周神经鞘瘤的CT和MRI表现  

CT and MRI features of malignant peripheral nerve sheath tumor of children

作　　者：刘志敏[1] 宋蕾[1] 高军[1] 于彤[1] 尹光恒[1] 蒋玲[1] 彭芸[1] 胡克非 

机构地区：[1]首都医科大学附属北京儿童医院影像中心,100045 [2]安徽省儿童医院放射科

出　　处：《中华放射学杂志》2015年第9期690-693,共4页Chinese Journal of Radiology

基　　金：国家自然科学基金（31271161）;首都临床特色应用研究（Z141107002514005）

摘　　要：目的分析儿童恶性外周神经鞘瘤（MPNsT）的CT和MRI特点，提高对本病的认识。方法回顾性分析16例经手术病理证实的MPNST的CT和MRI表现。男8例、女8例；年龄0．3—11．0岁，中位年龄2．5岁。16例均进行了CT平扫，其中8例行CT增强扫描，3例进行了MR检查。2名高年资儿童影像医师共同对图像进行分析，并协商达成一致意见。结果16例瘤灶位于颈部3例、腰背部5例、腹盆腔3例、足部2例、左侧锁骨下1例、右纵隔1例、右侧眼眶1例。15例呈肿块型，1例呈弥漫生长。CT表现：15例肿块型平扫呈实性为主（8例）或囊实性（7例）。增强扫描实性成分呈中度以上强化、渐进延迟强化，囊性成分未见明显强化。1例弥漫生长病变位于左侧颈部，与周围组织分界不清。MRI表现：2例肿块型MRI表现为T1WI呈等低信号，T2WI呈等高信号，T2WI囊性成分明显高信号，增强后有明显不均匀强化。1例弥漫生长病变MRI表现：T1WI呈稍低信号，T2WI呈等稍高信号，并明显强化。16例均呈不同程度侵袭性生长，11例伴多脏器转移及复发，4例合并神经纤维瘤病I型，并脊柱侧弯。结论恶性外周神经鞘瘤CT及MRI表现具有一定特征改变，并能显示其侵袭性表现及多脏器转移表现，有利于明确诊断并协助临床医生制定治疗方案。Objective To analyze CT and MR features of malignant peripheral nerve sheath tumor （MPNST）of children. Methods Sixteen patients with histologically proven MPNST were retrospectively reviewed.There were 8 male and 8 female, ages from 0.3 to 11.0 years, and median age was 2.5 years. Sixteen cases were performed with CT plain scan, and eight cases with CT enhancement scans, and three with MR examination. The imaging data were analyzed by two highly experienced doctors and obtained agreements after mutual consultation. Results Among 16 cases, 3 cases were located at neck, 5 cases at waist and back,3 cases at abdominal and pelvic, 2 cases at foot, 1 case at left clavicle, 1 case at right mediastinum, 1 case at right orbit. Fifteen cases appeared as solid masses and 1 case showed a diffuse growth. CT plain scan showed 8 cases were solid-appearing masses and 7 cases were cystic-solid mass. Enhanced CT showed enhancement of solid component was moderate to marked and gradually delayed enhanced while cystic component had no any enhancement. One was located on the left side of the neck and appeared as diffuse growth. Two cases of solid mass type appeared as hypo-intensity on T1WI and hyper-intensity on T2WI, and obviously high signal intensity of cystic component on T2WI, and with significantly heterogeneous enhanced.One case with diffuse growth appeared as hypo-intensity on T1WI and slightly hyper-intensity on T2WI, and with significantly enhanced. Sixteen cases appeared as invasive growth, 11 caseswith multiple organic metastases and recurrences, and 4 cases with neurofibromatosis type I and scoliosis.Conclusion CT and MR appearances of MPNST have certain characteristic features, and can demonstrateaggressive performance and multiple organic metastases, which is helpful for definite diagnosis and treatment plan.

关 键 词：神经鞘瘤 儿童 体层摄影术 X线计算机 磁共振成像 

分 类 号：R739.4[医药卫生—肿瘤] R730.44[医药卫生—临床医学] R445.2