侵犯视神经的视网膜母细胞瘤患儿465例预后分析  

Prognostic observation of 465 patients with retinoblastoma with optic nerve invasion

作　　者：王一卓[1] 黄东生[1] 史季桐[2] 马建民 赵军阳[2] 李彬[2] 项晓琳[2] 胡慧敏[1] 周燕[1] 

机构地区：[1]首都医科大学附属北京同仁医院儿科,100730 [2]首都医科大学附属北京同仁医院眼科,100730

出　　处：《中华儿科杂志》2015年第2期109-113,共5页Chinese Journal of Pediatrics

基　　金：首都医科大学附属北京同仁医院科研骨干培育基金（2014-VJJ-GGL-042）

摘　　要：目的 观察眼球摘除术后病理侵犯视神经的视网膜母细胞瘤（RB）患儿的治疗及预后情况.方法 回顾性分析北京同仁医院2006年1月至2013年12月临床确诊为单眼RB,眼球摘除后病理诊断侵犯视神经的465例患儿的病例资料,根据其病理侵犯程度分为4级：1级为肿瘤细胞侵犯视乳头;2级为侵犯筛板;3级为侵犯筛板之后,未到视神经断端;4级为侵犯视神经断端.1级和2级统称为侵犯筛板前视神经,视为病理学低风险因素;3级和4级统称为侵犯筛板后视神经,视为病理学高风险因素.其他病理学高风险因素包括侵犯大范围脉络膜、巩膜、前房、虹膜和睫状体.将患儿分为合并及不合并其他病理学高风险因素组.分别给予相应的治疗并观察其预后.随访时间6个月～7年,平均随访时间3年7个月.结果 465例患儿中男279例,女186例.右眼260例,左眼205例.从发现症状到就诊的时间为1d～2年,平均2.7个月.465例患儿中25例死亡,总生存率为94.6％.侵犯筛板前视神经的患儿有338例,2例复发死亡,生存率为99.4％ （336/338）;侵犯筛板后视神经的患儿有127例,23例复发死亡,生存率为81.9％ （104/127）;二者差异有统计学意义（χ2=52.299,P=0.000）.1～4级视神经侵犯患儿的病死率分别为0.4％、1.0％、8.7％、60.9％;465例患儿中379例不合并其他病理学高风险因素,8例死亡,病死率为2.1％;86例合并其他病理学高风险因素,17例死亡,病死率为19.8％;二者差异有统计学意义（χ2=42.955,P=0.000）.侵犯筛板前视神经的338例患儿中304例不合并其他高风险因素,复发死亡O例;34例合并其他高风险因素,2例复发死亡,病死率为5.9％;二者差异有统计学意义（P =0.010）.侵犯筛板后视神经的127例患儿中76例不合并其他高风险因素,9例复发死亡,病死率11.8％;51例合并其他高风险因素,14例复发死亡,病死率27.5％;二者差异有统计学意义（χ2 =5.014,P =0.025）.�Objective To observe the treatment and prognosis of optic nerve invasion in retinoblastoma （RB）.Method The children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2013 in our hospital were recruited.Tumor extension into the optic nerve were disclosed.Optic nerve involvement was classified into four grades according to the degree of invasion.Grade Ⅰ is superficial invasion of the optic nerve head only,grade Ⅱ is involvement up to and including the lamina cribrosa,grade Ⅲ is involvement beyond the lamina cribrosa,and grade Ⅳ is involvement up to and including the surgical margin.Grade Ⅰ and Ⅱ are called invasion of the optic nerve before the sieve plate.Grade Ⅲ and Ⅳ are called invasion of the optic nerve after the sieve plate.Other high-risk factors included extensive invasions of the choroid,sclera,anterior chamber,iris,and ciliary body.They were divided into two groups according to whether the merger of other high histopathologic risk factors.Treatment was delivered accordingly,and the prognosis of different degrees of optic nerve invasion was observed.The subjects were followed up for 6 months to 7 years （average：43 months）.Result There were 465 subjects in this study,including 279 boys and 186 girls.The right eye was affected in 260 patients and the left eye in 205 patients.The average time from onset of symptoms to visit was 2.7 months （range 1 day-24 months）.Twenty-five patients died,resulting in an overall survival rate of 94.6%.The mortality rate of patients with optic nerve involvement with grade Ⅰ was 0.4%,grade Ⅱ was 1.0%,grade Ⅲ was 8.7% was and grade Ⅳ was 60.9%.Of the 338 with invasion of the optic nerve before the sieve plate,two died of recurrence,with a survival rates of 99.4% （336/338）.Of the 127 patients who had invasion of the optic nerve after the sieve plate,twenty-three died of recurrence,with a survival rate of 81.9% （104/127）,the difference was statistically significant （χ2 =52.299,P =0.0

关 键 词：视网膜母细胞瘤 视神经 预后 

分 类 号：R739.7[医药卫生—肿瘤]