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

作　　者：赵倩[1] 金眉[1] 赵文[1] 王希思[1] 段超[1] 马晓莉[1] 苏雁[1] ZHAO Qian;JIN Mei;ZHAO Wen;WANG Xisi;DUAN Chao;MA Xiaoli;SU Yan(Medical Oncology Department,Pediatric Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing Key Laboratory of Pediatric Hematology Oncology,National Key Clinical Discipline of Pediatric Oncology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China)

机构地区：[1]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,北京100045

出　　处：《中国小儿血液与肿瘤杂志》2024年第5期364-367,372,共5页Journal of China Pediatric Blood and Cancer

基　　金：北京市自然科学基金(项目编号:7222054);北京市卫生健康委员会研究型病房(项目编号:BCRW202101)。

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。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 

分 类 号：R739.4[医药卫生—肿瘤]