北京儿童医院2009年至2017年眼内视网膜母细胞瘤患儿死亡原因分析  

Cause of death of children with intraocular retinoblastoma in the Beijing Children's Hospital 2009-2017

作　　者：张诗晗 马晓莉[2] 张诚玥[2] 白大勇[2] 崔燕辉[2] 苏雁[1] 张大伟[1] 王希思 段超[1] 赵军阳[2] 金眉[1] Zhang Shihan;Ma Xiaoli;Zhang Chengyue;Bai Dayong;Cui Yanhui;Su Yan;Zhang Dawei;Wang Xisi;Duan Chao;Zhao Junyang;Jin Mei(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 Ocology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China;Department of Ophthalmology,Beijing Children's Hospital,Capital Medical University,National Center for Children’s Health,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肿瘤中心肿瘤内科北京市儿童血液肿瘤重点实验室儿科重大疾病研究教育部重点实验室,北京100045 [2]首都医科大学附属北京儿童医院眼科国家儿童医学中心,北京100045

出　　处：《中华眼底病杂志》2022年第3期182-186,共5页Chinese Journal of Ocular Fundus Diseases

基　　金：国家科技重大专项(2017ZX09304029)。

摘　　要：目的:观察并分析眼内期视网膜母细胞瘤(RB)死亡患儿的临床特征。方法:回顾性临床分析。2009年至2017年于北京儿童医院接受治疗后死亡的眼内期RB患儿14例23只眼纳入研究。其中,男性7例10只眼,女性7例13只眼;单眼5例,双眼9例。患儿年龄(17.2±15.5)个月。所有患儿均行数字化广域眼底成像系统检查。参照眼内期RB国际分期标准对RB进行分期。23只眼中,B期1只眼,C期2只眼,D期12只眼,E期8只眼。患儿接受的治疗手段包括全身化学药物长春新碱、依托泊苷和卡铂方案(VEC方案)治疗、眼球摘除手术、玻璃体切割手术。回顾分析患儿基本情况、就诊年龄、诊断时间、就诊原因、病理诊断、治疗及主要死亡原因。结果:14例患儿中,首发症状表现为白瞳症12例;眼红1例;斜视1例。行全身VEC方案治疗1~6个疗程;眼球摘除手术5例,其中3例获取到病理标本行组织病理学检查;玻璃体切割手术治疗3例。行组织病理学检查的3例中,累及巩膜和视神经、视神经、视盘各1例。肿瘤转移和(或)颅内病灶死亡7例(50.0%,7/14),患儿中位生存期19个月。因治疗相关死亡4例(28.6,4/14),包括化学药物治疗相关副作用死亡3例,眼球摘除手术后器官衰竭死亡1例;患儿中位生存期3.5个月。早期放弃治疗死亡3例(21.4%,3/14);患儿中位生存期15个月。结论:颅内转移是眼内期RB患儿死亡的主要原因。Objective To observe and analyze the clinical characteristics of children who died of intraocular retinoblastoma(RB).Methods A retrospective clinical study.Fourteen children(23 eyes)with intraocular RB who died after receiving treatment in Beijing Children's Hospital from 2009 to 2017 were included in the study.Among the children,there were 7 males(10 eyes)and 7 females(13 eyes);5 had unilateral and 9 had bilateral tumor.Age were 17.2±15.5 months.All children underwent RetCam examination.RB was staged according to the international intraocular RB classify.Among the 23 eyes,1 eye was in stage B,2 eyes were in stage C,12 eyes in stage D,and 8 eyes in stage E.Treatment methods included a systemic(vincristine,etoposide and carboplatin)chemotherapy(VEC chemotherapy),enucleation surgery,and vitrectomy.The basic conditions including age,time of diagnosis,pathological diagnosis,treatment and main causes of death were retrospectively analyzed.Results Among the 14 cases,the first symptom was leukemia in 12 cases,red eye in 1 case,and squintin in 1 case.Systemic VEC chemotherapy was used for 1-6 courses of treatment;5 cases were enucleated,3 cases underwent histopathological examination;3 cases were treated with vitrectomy.Among the 3 cases who underwent histopathological examination,the sclera and optic nerve,optic nerve and optic disc were invasted respectively.Seven patients died of tumor metastasis and/or intracranial lesions(50.0%,7/14);the median survival time was 19 months.Four patients died of treatment(28.6%,4/14),including 3 patients died of chemotherapy-related side effects,and 1 died of organ failure after enucleation surgery(7.1%);the median survival time was 3.5 months.Early abandonment of treatment died in 3 cases(21.4%,3/14);the median survival time was 15 months.Conclusion Intracranial metastasis is the main cause of death in children with intraocular RB.

关 键 词：视网膜母细胞瘤 死亡原因 抗肿瘤联合化疗方案 眼球摘除术 玻璃体切除术 

分 类 号：R739.7[医药卫生—肿瘤]