摘要
背景婴儿期起病的中枢神经系统(CNS)肿瘤临床罕见,国内少见报道。目的探讨婴儿期起病的CNS肿瘤患儿的临床特征、病理类型、治疗结局及预后的影响因素。设计回顾性队列研究。方法以2011年6月至2019年12月婴儿原发CNS肿瘤病例为队列起点,采用多学科诊疗模式手术联合化疗,以2020年3月31日为随访终点,采集性别、起病年龄、有无转移、肿瘤部位、肿瘤级别、肿瘤直径、切除程度、是否化疗作为生存率(OS)和无事件生存率(EFS)的预后影响因素。主要结局指标OS和EFS的预后影响因素。结果52例初诊婴儿原发CNS肿瘤进入本文分析,男36例,女16例;诊断时中位年龄6.7(0~12)个月,起病年龄<6月龄18例,其中新生儿期起病2例。幕上33例,幕下17例,脊髓2例;肿瘤直径<5 cm 26例,≥5 cm 19例,不详7例。行手术治疗44例中,全切/次全切39例(89%),部分切除4例,非开颅活检1例,起病即转移4例。经临床和影像学诊断,评估手术风险高和经济原因等未手术治疗8例,于诊断后0.9(0~1.24)个月死亡。低级别肿瘤24例,高级别肿瘤20例。脉络丛肿瘤17例,胶质瘤11例,胚胎性肿瘤13例,非成熟畸胎瘤2例,先天性造釉细胞型颅咽管瘤1例。手术后化疗24例,复发或进展11例(其中死亡10例),EFS 13例,其中有事件存活1例。手术后未化疗20例,1例复发或进展后死亡,EFS 19例。COX回归分析显示,肿瘤非全切较肿瘤全切/次全切OS和EFS的HR分别为5.7(95%CI:1.408~23.115)和5.1(95%CI:1.260~20.731);高级别肿瘤较低级别肿瘤婴儿OS和EFS的HR分别为18.0(95%CI:2.222~146.5897)和8.3(95%CI:1.687~40.530);肿瘤部位幕下较幕上婴儿OS和EFS的HR分别为4.2(95%CI:1.563~11.291)和4.9(95%CI:1.996~12.216)。结论婴儿原发CNS肿瘤低级别肿瘤预后良好,高级别肿瘤如非典型畸胎瘤/横纹肌样瘤、伴多层菊形团胚胎性肿瘤及松果体母细胞瘤预后差,肿瘤非全切、高级别肿瘤和幕下肿瘤为影响OS和EFS�
Background Central nervous system(CNS)tumors in infancy are rare and very few reports are available in China.Objective To investigate the clinical features,pathological spectrum,survival outcome and prognostic factors in infants with CNS tumors.Design Retrospective cohort study.Methods The starting point of the cohort was from June 2011 to December 2019,and infants under the age of 12 months with the initial diagnosis of primary CNS tumors were enrolled and treated by a multidisciplinary model of surgery in combination with chemotherapy.The end of follow⁃up was March 31,2020.Metastasis,tumor site,tumor grade,tumor diameter,degree of resection,and chemotherapy were used as prognostic factors for overall survival(OS)and event⁃free survival(EFS).Main outcome measures Prognostic factors for OS and EFS.Results Among 52 patients included in this study,there were 36 males and 16 females.The median age of onset was 6.7(range,0-12)months,less than 6 months in 18 cases,including 2 cases of neonatal onset.Thirty⁃three cases were supratentorial,17 cases were infratentorial,and 2 cases were in spinal cord.The tumor diameter was<5 cm in 26 cases,≥5 cm in 19 cases,and unknown in 7 cases.A total of 44 cases(84.6%)underwent surgical resection of tumors with 39 cases(89%)under gross total/near total resection,five cases under partially resection,and 1 case of non⁃invasive brain biopsy.At the time of diagnosis,4 patients experienced metastasis(M+).Eight cases gave up due to economic reasons or high surgical risk and all died 0.9(0-1.24)months after diagnosis.Twenty⁃four cases of low⁃grade tumors(WHO grade I+Ⅱ)and 20 high⁃grade tumors(WHO gradeⅢ+Ⅳ)were confirmed.There were 17 cases of choroid plexus tumor,11 cases of glioma,13 cases of embryonal tumors,2 cases of atypical teratoma,and 1 case of craniopharyngioma.Twenty-four patients(46%)received chemotherapy after surgery among whom eleven cases relapsed or experienced progression including 10 cases of death,and 13 cases were in event⁃free survival and includ
作者
李苗
朱帅
支天
谢瑶
孙艳玲
王淑梅
杜淑旭
龚小军
金眉
葛明
宫剑
赵卫红
刘嵘
黄东生
孙黎明
武万水
马晓莉
LI Miao;ZHU Shuai;ZHI Tian;XIE Yao;SUN Yanling;WANG Shumei;DU Shuxu;GONG Xiaojun;JIN Mei;GE Ming;GONG Jian;ZHAO Weihong;LIU Rong;HUANG Dongsheng;SUN Liming;WU Wanshui;MA Xiaoli(Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China,Department of Pediatrics;Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China,Department of Pharmacy;Children′s Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China,Department of Oncology;Children′s Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China,Department of Neurosurgery;Department of Pediatrics,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China;Department of Pediatrics,Peking University First Hospital,Beijing 100034,China;Department of Pediatric Neurosurgery,Beijing Tiantan Hospital,Capital Medical University,Beijing 100070,China;Department of Hematology,Children′s Hospital,Capital Institute of Pediatrics)
出处
《中国循证儿科杂志》
CSCD
北大核心
2021年第6期407-413,共7页
Chinese Journal of Evidence Based Pediatrics
关键词
中枢神经系统肿瘤
婴儿
治疗
多中心
Central nervous system tumor
Infant
Treatment
Multicenter study
