中枢神经系统非典型畸胎样/横纹肌样瘤6例临床病理分析  

Atypical teratoid / rhabdoid tomor in central nervous system: a clinicopathological analysis of six cases

作　　者：岳振营[1] 董艳光[1] 胡营营[1] 张洪艳[1] 徐滨[1] 李文雪[1] YUE Zhen-ying DONG Yan-guang HU Ying-ying ZHANG Hong-yan;XU Bin;LI Wen-xue(Dapartment of Pathology, the Central Hospital of Shengli Oilfield, Dongying 257000, Chin)

机构地区：[1]东营市胜利油田中心医院病理科,山东东营257000

出　　处：《诊断病理学杂志》2016年第9期655-658,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨中枢神经系统非典型畸胎样/横纹肌样瘤（AT/RT）的临床病理特征、免疫组化及鉴别诊断。方法分析6例中枢神经系统AT/RT的临床病理和影像学资料,对肿瘤标本进行常规病理组织学和免疫组化检查,并结合相关文献,探讨其临床病理特征。结果男性4例,女性2例,年龄2～11岁,平均6.7岁。影像学检查显示肿瘤均位于幕上,其中2例位于脑室内。组织学上,肿瘤由横纹肌样细胞及间叶组织或原始神经外胚层成分构成。免疫组化示vimentin、EMA、S-100、NSE和Syn（＋）,且所有病例均INI1蛋白表达缺失。结论 AT/RT是好发于儿童的罕见的高度恶性胚胎性肿瘤,临床表现为颅内占位性病变的相关症状或体征,病理上肿瘤细胞具有多能分化组织形态和免疫表型;该肿瘤的诊断依赖病理组织学及免疫组化检查,预后较差。Purpose To investigate the clinicopathologic features,immunohistochemical stain,diagnosis and differential diagnosis of atypical teratoid tumor / rhabdoid tomor（ AT / RT） in the central nervous system. Methods Six cases of AT / RT of the central nervous system were studied by hematoxylin-eosin staining, cytochemical and the immunohistochemical stains with combination of clinicopathologic features,imaging data and review of the literature.Results The patients included 4 males and 2 females,aged 2 to 11 years（ mean 6. 7 years）; imaging showed that tumors located above the tentorium,including 2 cases in intraventricular cavity. Histologically,the tumor was composed of rhabdoid tumor cells and mesenchymal tissue and / or primitive neuroectodermal composition without epithelial differentiation. Immunohistochemical staining showed vimentin,EMA,S-100,NSE and synaptophysin were positive,and INI1 protein expression was deleted in all cases. Conclusion AT / RT is a rare and highly malignant embryonal tumor most commonly occurred in children. The clinical manifestation of the lesion includes intracranial mass related symptoms,and the tumor has the morphology and immunohistochemical phenotype of multiple differentiation potential. It is important for correct dignosis to use immunohistochemical studies. Surgery is the main treatment method with chemotherapy or peripheral stem cell therapy,which can prolong survival time,but the prognosis is very poor.

关 键 词：非典型畸胎样/横纹肌样瘤 免疫组化 颅内肿瘤 

分 类 号：R729[医药卫生—儿科]