儿童脉络丛癌12例临床特征及生存分析  

Clinical features and survival analysis of 12 children with choroid plexus carcinoma

作　　者：李苗[1] 杜淑旭[1] 王淑梅[2] 龚小军[1] 任思其[1] 张金[1] 张臻 孙华颖 孙艳玲[1] 邱晓光[3] 刘巍[4] 李春德[4] 武万水[1] 孙黎明[1] LI Miao;DU Shuxu;WANG Shumei;GONG Xiaojun;REN Siqi;ZHANG Jin;ZHANG Zhen;SUN Huaying;SUN Yanling;QIU Xiaoguang;LIU Wei;LI Chunde;WU Wanshui;SUN Liming(Beijing Shijitan Hospital,Capital Medical University,Department of Pediatrics,Beijing 100038,China;Beijing Shijitan Hospital,Capital Medical University,Department of Pharmacy,Beijing 100038,China;Beijing Tiantan Hospital,Capital Medical University,Department of Radiotherapy,Beijing 100070,China;Beijing Tiantan Hospital,Capital Medical University,Department of Pediatric Neurosurgery,Beijing 100070,China)

机构地区：[1]首都医科大学附属北京世纪坛医院儿科,北京100038 [2]首都医科大学附属北京世纪坛医院药剂科,北京100038 [3]首都医科大学附属北京天坛医院放疗科,北京100070 [4]首都医科大学附属北京天坛医院小儿神经外科,北京100070

出　　处：《中国循证儿科杂志》2023年第2期124-128,共5页Chinese Journal of Evidence Based Pediatrics

基　　金：国家自然科学基金资助项目:No.81872926;北京世纪坛医院青年基金:2022-q19。

摘　　要：背景儿童脉络丛癌(CPC)临床罕见,国内报道较少。目的 探讨儿童CPC的临床特征、治疗及结局。设计病例系列报告。方法 回顾性分析首都医科大学附属北京世纪坛医院儿科于2017年1月至2022年10月收治的手术后病理确诊的CPC患儿,随访截至2022年12月31日。截取患儿性别、诊断年龄、临床表现、治疗和随访情况,生存数据采用Kaplan-Meier法分析。主要结局指标总体生存期(OS)和无进展生存期(PFS)。结果 12例CPC患儿纳入分析,男4例,女8例;中位诊断年龄29.7(5.8~119.6)个月,起病年龄<3岁8例;肿瘤直径≥5 cm 8例,<5 cm 4例;肿瘤位于幕上9例,幕下3例;肿瘤位于脑室系统6例,脑室外累及脑实质6例;起病时发现播散转移2例,无转移10例。12例均接受肿瘤切除手术,全切8例,近全切4例。术后仅行化疗5例(42%),联合放射治疗及化疗7例(58%)。截至末次随访,8例出现肿瘤复发或进展,其中4例因肿瘤进展后死亡。平均OS(56.7±8.8)个月,1、3、5年OS率分别为(83.3±10.8)%、(66.7±13.6)%和(66.7±13.6)%。平均PFS(24.3±7.2)个月,1、3年PFS率分别为(41.7±14.2)%和(33.3±13.6)%。Kaplan-Meier单因素分析发现,肿瘤位于幕下3年OS低于幕上(χ^(2)=8.562,P=0.003);单纯化疗3年OS低于放化疗联合治疗(χ^(2)=8.488,P=0.004);不同性别、起病年龄(<3岁与3~18岁)、肿瘤直径(≥5 cm与<5 cm)、切除程度、有无转移、是否放化疗对3年PFS的影响差异均无统计学意义(P均>0.05)。结论 儿童CPC临床罕见,预后差,肿瘤位于幕下及单纯化疗为影响OS的不良预后因素。Background Choroid plexus carcinoma(CPC)is very rare in children,and there are few reports in China about this special cohort.Objective To investigate the clinical features,treatment and survival outcome in pediatric CPC patients.Design Case series report.Methods Children with CPC confirmed by pathology after tumor resection,who were admitted to pediatric department of Beijing Shijitan Hospital from January 2017 to October 2022,were retrospectively analyzed.The last follow-up was December 31,2022.The sex,age at diagnosis,clinical features,treatment and follow-up were collected.Kaplan-Meier method was adopted for survival analysis.Main outcome measures Overall survival(OS)and progression-free survival(PFS).Results Among the 12 included patients,four were males and eight were females.The median age at diagnosis was 29.7(range,5.8-119.6)months,with 8 cases less than 3 years old at the time of diagnosis,tumor diameter≥5cm in 8 cases,and<5cm in 4 cases.Nine cases were supratentorial,and 3 were infratentorial.The tumor was located in ventricular system in 6 cases,and external involvement of brain parenchyma in 6 cases.Two cases experienced metastases(M+)and 10 patients without metastasis(M0)at the time of diagnosis.All the patients received tumor resection with 8 cases under gross total resection(GTR)and 4 near total resection(NTR).Five(42%)cases received chemotherapy only and 7 patients(58%)were treated with both radiotherapy and chemotherapy after surgery.At the time of last follow-up,8 cases experienced tumor recurrence or progression,and 4 cases died.The mean OS were(56.7±8.8)months.The 1,3,5-year OS were(83.3%±10.8)%、(66.7%±13.6)%,and(66.7%±13.6)%,respectively.The mean PFS time were(24.3±7.2)months.The 1,3-year PFS were(41.7%±14.2)%and(33.3%±13.6)%,respectively.The Kaplan-Meier univariate analysis showed the 3-year OS was poorer in children with infratentorial tumors than those with supratentorial tumors(χ^(2)=8.562,P=0.003).And the 3-year OS was also lower in patients who received chemotherapy only th

关 键 词：脉络丛癌 儿童 治疗 

分 类 号：R739.4[医药卫生—肿瘤]