CAJ | 학술논문

目的结合1例左侧小脑脑桥角(CPA)区的恶性外周神经鞘膜瘤患者的临床资料,探讨罕见部位恶性外周神经鞘膜瘤的发生机制、临床、病理特点及治疗方法.方法回顾性分析1例左侧CPA区的恶性外周神经鞘膜瘤患者的临床资料.本患者临床表现为无明显诱因下出现反复头晕,左侧颜面部麻木,伴有左侧耳鸣、恶心,无头痛、呕吐.头颅MRI:左侧CPA区见一大小约3.0 cm×2.0 cm ×2.0 cm的团块状等T1长T2信号,其中可见囊变坏死区,周围无明显水肿,增强扫描病灶明显不均匀强化,听神经增粗不明显,可见轻微强化,第四脑室及脑桥明显受压,脑室系统无明显扩大,脑沟裂无明显增宽,中线结构居中.结果行左侧乙状窦后入路,显微镜下肿瘤切除.病理报告恶性外周神经鞘膜瘤.结论 CPA区的恶性外周神经鞘膜瘤少见,术后病理是诊断的主要依据,予以手术切除肿瘤的同时可合理选择放疗和药物治疗.
목적 결합1례좌측소뇌뇌교각(CPA)구적악성외주신경초막류환자적림상자료,탐토한견부위악성외주신경초막류적발생궤제、림상、병리특점급치료방법.방법 회고성분석1례좌측CPA구적악성외주신경초막류환자적림상자료.본환자림상표현위무명현유인하출현반복두훈,좌측안면부마목,반유좌측이명、악심,무두통、구토.두로MRI:좌측CPA구견일대소약3.0 cm×2.0 cm ×2.0 cm적단괴상등T1장T2신호,기중가견낭변배사구,주위무명현수종,증강소묘병조명현불균균강화,은신경증조불명현,가견경미강화,제사뇌실급뇌교명현수압,뇌실계통무명현확대,뇌구렬무명현증관,중선결구거중.결과 행좌측을상두후입로,현미경하종류절제.병리보고악성외주신경초막류.결론 CPA구적악성외주신경초막류소견,술후병리시진단적주요의거,여이수술절제종류적동시가합리선택방료화약물치료.
Objective To explore the mechanism,clinical manifestation,pathological features,diagnosis and treatment of malignant peripheral nerve sheath tumor in the rare location.Method The clinical date of a patient with malignant peripheral nerve sheath tumor were analyzed retrospectively.The clinical features were repeated dizziness and left side facial numbness without incentives,associated with left tinnitus,nausea,no headache and vomiting.MRI show a equate T1,long T2 lesion which had cystic change and necrosis in left cerebellopontine angle.The volume was 3.0 cm × 2.0 cm× 2.0 cm,without significant surrounding edema.The axial contrast - enhanced T1 - weighted image was non - uniform.Auditory nerve with slightly enhanced signal was not obviously thickening.Fourth ventricle and pons were compressed obviously.No midline shift and lateral ventricle expending were found.Result The tumor was dissected under microscope through suboccipital retrosigmoidal approach.Postoperative recovery was well.Pathological examination showed a malignant peripheral nerve sheath tumor.Conclusions Malignant peripheral nerve sheath tumor in cerebellopontine angle was seldom.Its diagnosis mainly depends on pathological examination.Radiotherapy and chemocherapy should be considered after surgery.