5例治疗中进展的儿童颅内非典型畸胎样/横纹肌样瘤
5 cases of intracranial atypical teratoid/rhabdoid tumor in children with progression during treatment
赵倩 1金眉 1赵文 1王希思 1段超 1马晓莉 1苏雁1
作者信息
- 1. 100045 北京,国家儿童医学中心,首都医科大学附属北京儿童医院,儿童肿瘤中心,肿瘤内科;儿童血液病与肿瘤分子分型北京市重点实验室;儿童肿瘤国家临床重点专科;儿科重大疾病研究教育部重点实验室
- 折叠
摘要
目的 总结治疗中进展的颅内非典型畸胎样/横纹肌样瘤(AT/RT)患儿的临床特征,分析进展及影响预后的相关因素.方法 回顾性分析治疗中进展的AT/RT患儿的临床资料.结果 5例治疗中进展的AT/RT患儿,男3例,女2例,中位发病年龄21.5(18-32)个月;4例病初为局限性病灶,1例伴有脊髓及脑膜受累;3例完整切除瘤灶,2例术后有残留;3例在进展后给予全脑或全中枢放疗.1例病初持续进展、脑疝并死亡,其余4例接受4-7个疗程化疗,平均进展时间5.4(2-10.5)个月,3例患儿于进展后2.8(0.5-3)个月死亡.结论 儿童ATRT预后差,即使强化疗期间仍进展迅速,完整切除及规律治疗可能改善预后,但远期生存率仍低.
Abstract
Objective Background To summarize the clinical features of intracranial atypical teratoid/rhabdoid tumor(AT/RT)with progress disease during treatment,and analyze the factors related to the progression and prognosis.Methods The clinical data and treatment outcomes of AT/RT patients were studied retrospectively.Results 5 cases were recruited in this study,including 3 boys and 2 girls.The median age of onset was 21.5(18-32)months.4 cases were localized disease,and 1 case had spinal cord and meninges involvement.The tumor was completely resected in 3 cases,and residual disease after operation in 2 cases.Three cases received cranial or craniospinal irradiation after progression.The tumor continued to progress in 1 case after partial resection,and the other 4 patients received 4-7 courses of chemotherapy,the average time of progression was 5.4(2-10.5)months.Except 1 case,which is still being treated after progression,the other 3 cases died 2.8(0.5-3)months after progression.Conclusions The prognosis of ATRT in children is poor.Even during intensive chemotherapy,ATRT progresses rapidly.Complete resection and regular treatment can improve the prognosis,while the long-term survival rate is still low.
关键词
非典型畸胎样/横纹肌样瘤/治疗中进展/预后/危险因素Key words
Atypical teratoidrhabdoid tumor/Progress during treatment/Prognosis/Risk factors引用本文复制引用
基金项目
北京市自然科学基金(7222054)
北京市卫生健康委员会研究型病房(BCRW202101)
出版年
2024
NETL
NSTL
万方数据