儿童颅内非典型畸胎样/横纹肌样瘤的临床分析  

Clinical Analysis of Atypical Rhabdomyoma/Teratoid Tumor in Children

作　　者：周刚 孙剑瑞[2] 杜伟[2] 麻来峰 ZHOU Gang;SUN Jianrui;DU Wei;MA Laifeng(Neurosurgery Department,the Third People’s Hospital of Henan Province,Zhengzhou 450052,China;Neurosurgery Department,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]河南省直第三人民医院神经外科,河南郑州450052 [2]郑州大学第一附属医院神经外科,河南郑州450052

出　　处：《河南医学研究》2024年第6期983-987,共5页Henan Medical Research

基　　金：河南省医学科技攻关计划省部共建重点项目(SBGJ202002108)。

摘　　要：目的探讨颅内非典型畸胎样/横纹肌样瘤(AT/RT)的影像学特征、临床表现及病理特点以提高对AT/RT的诊断及治疗认识。方法回顾性分析郑州大学第一附属医院收治的10例AT/RT患儿的临床资料,检索国内外文献进行总结分析。结果10例AT/RT中<2岁的患儿6例(60%),>2岁的患儿4例(40%);镜下全切除4例,次全切除6例。其中2例>3岁的患者接受放化疗处理。所有患者随访2~12个月,所有患者在随访期内复查头颅MRI均不同程度原位复发,其中1例发生脑膜转移及椎管内转移,1例伴发肺部转移,2例因原位复发和脑内转移而再次手术。结论AT/RT临床发病率低,临床多以颅内压增高为主要表现,治疗方法为手术联合放化疗,诊断仍依赖病理诊断,目前仍无标准放化疗方案,该肿瘤恶性程度高,易复发,预后差,病死率高。Objective To investigate the imaging,clinical and pathological features of atypical teratoid/rhabdomyoma(AT/RT)in order to improve the diagnosis and treatment of AT/RT.Methods Retrospective analysis of clinical data of 10 children with AT/RT admitted to the First Affiliated Hospital of Zhengzhou University,and retrieval of domestic and foreign literature for summary and analysis.Results There were 10 cases of AT/RT,including 6 patients under 2 years old(60%),4 patients over 2 years old(40%),4 cases underwent total resection under microscope,and 6 cases underwent subtotal resection.Two patients over 3 years old underwent radiotherapy and chemotherapy.All patients were followed up for 2 to 12 months,and all patients had varying degrees of in situ recurrence during the follow-up period.Among them,1 case had meningeal metastasis and intraspinal metastasis,1 case had concomitant lung metastasis,and 2 cases underwent reoperation due to in situ recurrence and brain metastasis.Conclusion The clinical incidence rate of AT/RT is relatively low.The clinical manifestations are mainly intracranial hypertension.Surgery is usually performed with concurrent chemoradiotherapy.The diagnosis is still dependent on pathological diagnosis.There is no standard chemotherapy and radiotherapy protocol.The tumor is highly malignant and easy to recur,with poor prognosis and high mortality.

关 键 词：非典型畸胎样/横纹肌样瘤 儿童 临床特征 

分 类 号：R739.41[医药卫生—肿瘤]