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中国循证儿科杂志

中国循证儿科杂志 ›› 2021, Vol. 16 ›› Issue (6): 407-413.

• 论著 •    下一篇

婴儿中枢神经系统肿瘤的生存和预后影响因素多中心回顾性队列研究

李，苗1a,7，朱帅2a,7，支天3,7，谢瑶4，孙艳玲1a，王淑梅1b，杜淑旭1a，龚小军1a，金眉2a，葛明2b，宫剑5赵卫红4，刘嵘6，黄东生3，孙黎明1a，武万水1a，马晓莉2a




  

  1. 1 首都医科大学附属北京世纪坛医院北京，100038，a 儿科，b 药剂科；2 首都医科大学附属北京儿童医院北京，100045 ，a 儿童肿瘤中心肿瘤内科，b 神经外科；3 首都医科大学附属北京同仁医院儿科北京，100730；4 北京大学第一医院儿科北京，100034；5 首都医科大学附属北京天坛医院小儿神经外科北京，100070；6 首都儿科研究所附属儿童医院血液科100020；7共同第一作者
  • 收稿日期:2022-01-05 出版日期:2021-12-25 发布日期:2021-12-25
  • 通讯作者: 武万水，马晓莉

Factors affecting the survival and prognosis of central nervous system tumors in infants: A multicenter retrospective cohort study

LI Miao1a,7, ZHU Shuai2a, ZHI Tian3,7, XIE Yao4, SUN Yanling1a, WANG Shumei1b, DU Shuxu1a, GONG Xiaojun1a, JIN Mei2a, GE Ming6, GONG Jian5, ZHAO Weihong4, LIU Rong6, HUANG Dongsheng3, SUN Liming1a, WU Wanshui1a, MA Xiaoli2a   

  1. 1 Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China：a Department of Pediatrics, b Department of Pharmacy; 2 Children′s Oncology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing 100045, China: a Department of Oncology, b Department of Neurosurgery; 3 Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China; 4 Department of Pediatrics, Peking University First Hospital, Beijing 100034, China; 5 Department of Pediatric Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China; 6 Department of Hematology, Children′s Hospital, Capital Institute of Pediatrics; 7 Cofirst authors
  • Received:2022-01-05 Online:2021-12-25 Published:2021-12-25
  • Contact: WU Wanshui, MA Xiaoli

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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：2.222~146.589 7）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的不良预后因素。

关键词: 中枢神经系统肿瘤, 婴儿, 治疗, 多中心

Abstract: 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 grade Ⅲ+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 including 1 case of survival with events. Among twenty cases (46%) who received no chemotherapy, 1 case died after relapse or progression, 19 cases were in eventfree survival. Multivariate COX regression analysis demonstrated that the hazardratio of OS and EFS of nongross total tumor resections was 5.7 （95% CI：1.408~23.115） and 5.1（95% CI：1.260~20.731）compared with gross total /near total tumor resections; 18.0（95%CI：2.222~146.5897）and 8.3（95%CI：1.687~40.530）were for high WHO grade tumors compared with lowgrade tumors; 4.2（95%CI：1.563~11.291）and 4.9（95%CI：1.996~12.216）were for infratentorial tumors compared with supratentorial cases. Conclusion: Primary lowgrade tumors in infancy have good prognosis. Highgrade tumors such as atypical teratoma /rhabdoid tumors, embryonic tumors with multiple rosettes and pineoblastoma have poor prognosis. Nongross total /near total tumor resections, highgrade tumor and infratentorial tumor location are poor prognostic factors for OS and EFS.

Key words: Central nervous system tumor, Infant, Treatment, Multicenter study