颅内非典型畸胎瘤样/横纹肌样瘤1例报告并文献复习  

Intracranial atypical teratoid/rhabdoid tumor(a report of 1 case and review of literature)

作　　者：朱晓锋[1] 李绍山[1] 付强[1] 周庆九[1] 刘波[1] 郭正[1] 柳琛[1] 

机构地区：[1]新疆医科大学第一附属医院神经外科,乌鲁木齐830054

出　　处：《临床神经外科杂志》2016年第1期56-59,共4页Journal of Clinical Neurosurgery

基　　金：新疆维吾尔自治区自然科学基金(2012211A079);新疆医科大学第一附属医院自然科学青年基金(2015ZRQN15)

摘　　要：目的探讨颅内非典型畸胎瘤样/横纹肌样瘤(AT/RT)的临床特点,诊断及治疗,提高对其认识水平。方法回顾性分析1例经病理和免疫组化诊断为颅内非典型畸胎瘤样/横纹肌样瘤患者的临床资料,并进行相关文献复习。结果患者主要表现头痛、头晕伴恶心,渐进性加重;MRI示病灶位于右侧岛叶。显微镜下手术切除肿瘤,免疫组化检查证实为颅内非典型畸胎瘤样/横纹肌样瘤。术后患者行放化疗,存活期12个月。结论颅内非典型畸胎瘤样/横纹肌样瘤高度恶性,极具侵袭性,生长迅速,预后极差,临床和影像学表现缺乏特异性;病理组织学改变是确诊的主要依据;采用以手术为主的个体化综合治疗是关键。Objective To explore the clinical feature,diagnosis and treatment of intracranial atypical teratoid / rhabdoid tumor（AT/RT）,and raise the level of understanding.Methods The clinical data of one patient with intracranial AT / RT diagnosed by pathological and immunohistochemical examination were analyzed retrospectively,and the literature was reviewed.Results The main manifestations of patient were headache,dizziness with nausea and progressive aggravation.MRI showed lesions located on the right side of the insula.The tumor was surgical removed under microscope and was confirmed as intracranial AT/RT by Immunohistochemical examination.Postoperative patients received radiotherapy and chemotherapy with survival period of 12 months.Conclusions Intracranial AT/RT is highly malignant and aggressive,rapid growth and the prognosis is poor.The clinical and imaging findings lack of specificity,histologic change are the main basis of diagnosis,the use of predominantly surgery individualized comprehensive treatment is the key.

关 键 词：中枢神经系统 非典型畸胎瘤样/横纹肌样瘤 诊断 

分 类 号：R736.4[医药卫生—肿瘤]