婴儿期视网膜母细胞瘤217例临床特点及预后分析  

Clinical characteristics and prognosis analysis of 217 infant patients with retinoblastoma

作　　者：支天[1] 张伟令[1] 张谊[1] 黄东生[1] 王一卓[1] 胡慧敏[1] Zhi Tian;Zhang Weiling;Zhang Yi;Huang Dongsheng;Wang Yizhuo;Hu Huimin(Department of Pediatrics,Beijing Tongren Hospital,Capital Medical University,Beijing 100176,China)

机构地区：[1]首都医科大学附属北京同仁医院儿科,100176

出　　处：《中华实用儿科临床杂志》2021年第15期1143-1147,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：北京市医院管理局"登峰"人才培养计划(DFL20180201)。

摘　　要：目的探讨婴儿期视网膜母细胞瘤(RB)的临床特点及预后。方法收集2009年7月1日至2019年6月30日首都医科大学附属北京同仁医院儿科收治的217例(患眼335只)婴儿期RB患儿的临床资料,分析其综合治疗的临床疗效及预后。结果1.临床特点:217例婴儿期RB患者中,男129例,女88例,男女比例为1.47∶1.00;中位年龄6.06个月;单眼发病99例,双眼发病118例,双单眼发病比例1.19∶1.00,5例有RB家族史患儿均为双眼发病;首发症状以白瞳、瞳孔黄白反光为主(183例,84.3%),其次为斜视(18例,8.3%)。患眼335只,其中眼内期304只(90.7%),以D期多见(146只,43.6%);眼外期26只(7.7%),主要侵犯视神经和/或视神经断端;远处转移期5只(1.5%)。2.生存分析:随访至2020年3月31日,中位随访时间67个月,失访2例,复发21例,死亡19例,总体生存率为91.2%。经Kaplan-Meier生存分析,5年预计生存率为91.1%;其中眼内期生存率96.2%,眼外期73.1%,5例远处转移期均死亡,差异有统计学意义(χ^(2)=7.492,P<0.001);单眼发病生存率(95.9%)、双眼发病生存率(87.3%)比较,差异有统计学意义(χ^(2)=4.335,P=0.023)。3.眼摘及保眼情况:217例患儿保眼率为68.9%,A、B、C期的保眼率为100.0%,D期的保眼率为80.1%,E期保眼率为35.1%,三者比较差异有统计学意义(χ^(2)=6.573,P=0.004)。化疗前行眼摘手术的患儿35例(其中D期6例,E期11例),化疗后眼摘67例(其中D期22例,E期36例),在化疗前后D、E期的眼摘率比较差异有统计学意义(χ^(2)=6.076,P=0.012)。4.不良反应:根据世界卫生组织化疗不良反应分级,0级26例(12.0%),Ⅰ级98例(45.1%),Ⅱ级59例(27.2%),Ⅲ级23例(10.6%),Ⅳ级11例(5.1%),主要表现为化疗后骨髓抑制(132例),未出现第二肿瘤,仅4例出现一过性听力异常,并在随后的复查中恢复正常。结论婴儿期RB具有其自身特点,不同眼别、不同临床分期等因素均可影响患儿预后,经治疗可有效提高患儿的生存率、降低其眼球�Objective To investigate the clinical characteristics and prognosis of infants with retinoblastoma(RB).Methods The clinical data of 217(335 affected eyes)infantile RB patients admitted to the Department of Pediatrics,Beijing Tongren Hospital,Capital Medical University from July 1,2009 to June 30,2019 were collected for analyzing the clinical efficacy and prognosis after comprehensive treatment.Results(1)Clinical characteristics:217 infantile RB patients included 129 males and 88 females,with the ratio of male to female being 1.47∶1.00;the median age was 6.06 months;there was monocular disease in 99 cases,and binocular disease in 118 cases,with the incidence ratio of binocular to monocular disease being 1.19∶1.00;all the 5 cases with family history of RB had binocular disease;the first symptoms included white pupils and yellow-white reflections in pupils(183 cases,84.3%),followed by strabismus(18 cases,8.3%).There were 335 affected eyes,of which 304 counts were in the intraocular stage(90.7%),most commonly in stage D(146 counts,43.6%);26 counts(7.7%)in the extraocular stage,mainly invading the optic nerve and/or optic nerve stump;5 counts(1.5%)in the metastasis stage.(2)Survival analysis:the medical follow-up continued to March 31,2020,with a median follow-up time of 67 months.There were 2 cases with a loss of follow-up,21 relapsed cases,and 19 death cases,with the overall survival rate being 91.2%.According to Kaplan-Meier survival analysis,the expected 5-year survival rate was 91.1%;the survival rate was 96.2%in the intraocular stage and 73.1%in the extraocular stage.All 5 cases died during the distant metastasis stage,and the difference was statistically significant(χ^(2)=7.492,P<0.001);there was also a statistical difference in the survival rate between the monocular disease(95.9%)and binocular disease(87.3%)(χ^(2)=4.335,P=0.023).(3)Eyeball removal and eye protection:the eye protection rate of 217 children was 68.9%,100.0%in stage A,B and C,80.1%in stage D and 35.1%in stage E,which showed significant dif

关 键 词：视网膜母细胞瘤 婴儿 化疗 预后 

分 类 号：R739.7[医药卫生—肿瘤]