北京儿童医院视网膜母细胞瘤3年诊断治疗效果总结
북경인동의원시망막모세포류3년진단치료효과총결
Treatment and short-term effect analysis of retinoblastoma in Beijing Children's Hospital for 3-year results
  • 万方数据
    中华实用儿科临床杂志 중화실용인과림상잡지
    Journal of Applied Clinical Pediatrics
    2014年 15期 1141-1144 ,共4页

    马晓莉%赵军阳%金眉%崔洁%白大勇%张大伟%崔燕辉%吴倩%于刚

    마효리%조군양%금미%최길%백대용%장대위%최연휘%오천%우강

    眼内期视网膜母细胞瘤%近期疗效%北京儿童医院 眼內期視網膜母細胞瘤%近期療效%北京兒童醫院
    안내기시망막모세포류%근기료효%북경인동의원
    Intraocular retinoblastoma%Short-term effect%Beijing Children's Hospital
    目的 建立多学科联合模式,应用全身化疗和局部治疗的综合治疗策略对眼内期视网膜母细胞瘤(RB)患儿进行诊治,评价该方法对保存RB患儿眼球、视力以及改善生存率的效果.方法 总结2009年11月至201 1年11月在首都医科大学附属北京儿童医院血液肿瘤中心诊治的全部眼内期RB患儿,分析临床资料和治疗情况,全部患儿随访至2012年12月31日.RB的诊断标准、分期、临床危险度分组、治疗方案和疗效标准均参照RB国际分类系统.化疗方案为改良的加拿大多中心RB 2003方案,同时应用810 nm及532 nm激光、冷凝技术进行局部治疗.通过随访收集患儿眼球摘除及生存质量的情况,同时在眼底镜检查下评估疗效及转移的风险.结果 共127例(180只眼),男68例,女59例,双眼51例,单眼78例(右41例,左37例).在双眼51例(102只眼)中,E期26只,D期52只,C期1 1只,B期11只,A期2只,D期伴视网膜脱落9只,E期伴视网膜脱落5只.单眼78例中,E期31例,D期47例,单眼中 D期伴视网膜脱落7例,E期伴视网膜脱落7例.年龄2 ～ 84个月(中位年龄17个月,其中双眼15.8个月,单眼23.2个月).除1例患儿化疗2个疗程后死于颅内出血,随访时间为2个月外,其他患儿随访时间6 ～38个月(中位时间29.2个月),总化疗疗程为1～11个疗程(平均5.2个疗程).共眼球摘除76只眼,其中直接眼球摘除32只,化疗后眼球摘除44只.眼球摘除后病理显示有高危因素10例(10只眼)(13％),其中前房种植4只眼,筛板后视神经受累(断端阴性)6只眼,葡萄膜后浸润(包括脉络膜受累)4只眼.单一危险因素3只眼,7只眼同时存在2种以上危险因素.在药物不良反应观察中,明确为卡铂致听力损伤5例,依托泊苷致皮疹3例.1例并发脓毒症.免疫指标恢复时间为停化疗至少6个月.共发生事件8例,其中失访1例为单眼患儿,化疗4个疗程后回当地医院治疗.7例死亡患儿包括2例拒绝眼球摘除者.其他死亡患儿为:单眼2例,分别为1例化疗3个疗程,眼球摘除2个月后颅内转移死亡;1例于化疗1个疗程,眼球摘除6个月后颅内进展放弃治疗,死亡.双眼2例,分别为1例停化疗11个月后出现颅内进展放弃治疗,死亡;1例在单侧眼球摘除后脏器功能衰竭死亡.其余患儿均存活至今,Kaplan-Meier进行单因素生存分析,中位随访时间29个月,生存率为93.7％.结论 肿瘤内科联合眼科,应用VEC方案(即长春新碱+卡铂+依托泊苷)结合激光、冷凝技术进行RB治疗,使眼内期RB患儿的近期疗效明显改善,成功保眼50只.建议对于晚期的单眼RB,眼球摘除后化疗2～4个疗程可能为预防局部和远处转移的关键.颅内转移为主要的转移部位,也是死亡的主要原因,提示治疗中的头颅影像检查很重要.
    목적 건립다학과연합모식,응용전신화료화국부치료적종합치료책략대안내기시망막모세포류(RB)환인진행진치,평개해방법대보존RB환인안구、시력이급개선생존솔적효과.방법 총결2009년11월지201 1년11월재수도의과대학부속북경인동의원혈액종류중심진치적전부안내기RB환인,분석림상자료화치료정황,전부환인수방지2012년12월31일.RB적진단표준、분기、림상위험도분조、치료방안화료효표준균삼조RB국제분류계통.화료방안위개량적가나대다중심RB 2003방안,동시응용810 nm급532 nm격광、냉응기술진행국부치료.통과수방수집환인안구적제급생존질량적정황,동시재안저경검사하평고료효급전이적풍험.결과 공127례(180지안),남68례,녀59례,쌍안51례,단안78례(우41례,좌37례).재쌍안51례(102지안)중,E기26지,D기52지,C기1 1지,B기11지,A기2지,D기반시망막탈락9지,E기반시망막탈락5지.단안78례중,E기31례,D기47례,단안중 D기반시망막탈락7례,E기반시망막탈락7례.년령2 ～ 84개월(중위년령17개월,기중쌍안15.8개월,단안23.2개월).제1례환인화료2개료정후사우로내출혈,수방시간위2개월외,기타환인수방시간6 ～38개월(중위시간29.2개월),총화료료정위1～11개료정(평균5.2개료정).공안구적제76지안,기중직접안구적제32지,화료후안구적제44지.안구적제후병리현시유고위인소10례(10지안)(13％),기중전방충식4지안,사판후시신경수루(단단음성)6지안,포도막후침윤(포괄맥락막수루)4지안.단일위험인소3지안,7지안동시존재2충이상위험인소.재약물불량반응관찰중,명학위잡박치은력손상5례,의탁박감치피진3례.1례병발농독증.면역지표회복시간위정화료지소6개월.공발생사건8례,기중실방1례위단안환인,화료4개료정후회당지의원치료.7례사망환인포괄2례거절안구적제자.기타사망환인위:단안2례,분별위1례화료3개료정,안구적제2개월후로내전이사망;1례우화료1개료정,안구적제6개월후로내진전방기치료,사망.쌍안2례,분별위1례정화료11개월후출현로내진전방기치료,사망;1례재단측안구적제후장기공능쇠갈사망.기여환인균존활지금,Kaplan-Meier진행단인소생존분석,중위수방시간29개월,생존솔위93.7％.결론 종류내과연합안과,응용VEC방안(즉장춘신감+잡박+의탁박감)결합격광、냉응기술진행RB치료,사안내기RB환인적근기료효명현개선,성공보안50지.건의대우만기적단안RB,안구적제후화료2～4개료정가능위예방국부화원처전이적관건.로내전이위주요적전이부위,야시사망적주요원인,제시치료중적두로영상검사흔중요.
    Objective To establish a multidisciplinary approach to intraocular retinoblastomas (RB) and to diagnose and treat intraocular retinoblastoma (RB) by systemic and local chemotherapy and to evaluate the curative effect of the therapy for preservation of eyes of the RB patients,eye sight,and the improvement of the survival rate.Methods We summarized the data of intraocular RB and reviewed medical records of 127 children (180 eyes) presenting with clinically and/or histologically confirmed RB and treated at Hematology oncology Centre,Beijing Children' s Hospital (BCH) Affiliated to Capital Medical University from Nov.2009 to Nov.2011.Eligible eyes were classified according to the International Intraocular Retinoblastoma Classification (IIRC).Based on modified Canadian RB 2003 protocols,therapeutic modalities were enucleation,local control (810 nm and 532 nm laser therapy,cryotherapy) and chemotherapy.Chemotherapy consisted of vincristine,etoposide,and carboplatin (VEC).All patients were followed up to Dec 31,2012.Cases of enucleation and quality of life of children were collected by follow-up,as well as to assess the efficacy and risk of transfer under the ophthalmoscopy.Results There were 127 patients (180 eyes),68 male and 59 female,with a median age of 17 months (range 2 to 84 months)at initial diagnosis.Seventy-eight patients had unilateral RB,31 eyes of unilateral RB were classified as group E(7 eyes with detached retina),47 eyes as group D (7 eyes with detached retina).Fifty-one had bilateral RB(102 eyes),26 eyes of bilateral RB were classified as group E (5 eyes with detached retina),52 eyes as group D (9 eyes with detached retina),group C,B and A were 11,11 and 2,respectively.With a mean chemotherapy of 5.2 cycles (range 1 to 11),76 eyes were enucleated (42.0％).Thirty-two eyes with advanced intraocular disease were enucleated at the time of presentation.Enucleation were then recommended for 44 of those eyes.Overall,50 eyes (42.3％) were successfully preserved without the need for enucleation.Ten enucleated eyes (13％) showed high-risk pathologic features.At the time of follow-up of 29.2 (range 6 to 38) months,event occurred in 8 cases,one patient lost follow-up,7 cases were died.One hundred and nineteen patients (93.7％) were alive.Conclusions Chemotherapy using VEC regimen was possible and effective in preventing metastasis.We were able to salvage almost half of these eyes.It is suggested that the advanced unilateral RB can be enucleated and then given chemotherapy for 2-4 courses to prevent local or distal metastasis.Intracranial site is the main site of metastasis which is also the main cause of death,so cranial imaging examination is very important during treatment.
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