CAJ | 학술논문

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。
목적：분석인동악성외주신경초류（MPNST）적CT화MRI특점，제고대본병적인식。방법회고성분석16례경수술병리증실적MPNST적CT화MRI표현。남8례、녀8례；년령0.3～11.0세，중위년령2.5세。16례균진행료CT평소，기중8례행CT증강소묘，3례진행료MR검사。2명고년자인동영상의사공동대도상진행분석，병협상체성일치의견。결과16례류조위우경부3례、요배부5례、복분강3례、족부2례、좌측쇄골하1례、우종격1례、우측안광1례。15례정종괴형，1례정미만생장。CT표현：15례종괴형평소정실성위주（8례）혹낭실성（7례）。증강소묘실성성분정중도이상강화、점진연지강화，낭성성분미견명현강화。1례미만생장병변위우좌측경부，여주위조직분계불청。MRI표현：2례종괴형MRI표현위T1WI정등저신호，T2WI정등고신호，T2WI낭성성분명현고신호，증강후유명현불균균강화。1례미만생장병변MRI표현：T1WI정초저신호，T2WI정등초고신호，병명현강화。16례균정불동정도침습성생장，11례반다장기전이급복발，4례합병신경섬유류병Ⅰ형，병척주측만。결론악성외주신경초류CT급MRI표현구유일정특정개변，병능현시기침습성표현급다장기전이표현，유리우명학진단병협조림상의생제정치료방안。
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.