小脑脑桥角恶性外周神经鞘膜瘤一例并文献复习

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.

Diagnosis and treatment of malignant peripheral nerve sheath tumor in cerebellopontine angle : cast report and review of literature

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.