42例双眼眼内期视网膜母细胞瘤疗效分析

The clinical analysis of 42 cases with bilateral intraocular retinoblastoma

目的观察双眼眼内期视网膜母细胞瘤(RB)患儿全身化学药物治疗联合局部治疗的临床效果。方法总结我院2009年12月-2011年2月收治的42例RB患儿的临床资料。其中男22例,女20例。系统化疗采用长春新碱+足叶乙甙+卡铂(VEC)方案治疗,同时应用激光、冷凝技术进行局部治疗,对患儿的保眼率、化疗安全性进行评估。结果(1)中位年龄13个月,双眼发病患儿发病年龄较同期单眼患儿小,但未见显著统计学差异(P=0.075)。(2)29例患儿以白瞳为主要表现,占69%。(3)疗效与预后:患儿初诊时Group A期眼3只(3.6%);Group B期眼9只(10.7%);Group C期眼10只(11.9%);Group D期眼40只(47.6%);Group E期眼22只(26.2%)。眼球摘除患儿21人,化疗前后D及E期眼摘率有显著差异(P=0.016)。本组患儿保眼率:E期11只(50%);D期25只(62.5%);C期9只(90%);B期9只(100%);A期3只(100%)。总保眼率67.8%。本组患儿共有5例死亡(12%),4例为肿瘤进展,1例为化疗后颅内出血死亡。(4)化疗毒副作用:仅3例出现一过性听力异常并在随后的复查中双耳听力阈值恢复正常。结论未发现患儿存在明显的、不可逆的听力损害,提示本方案的安全性。对于双眼RB患儿,局部治疗联合化疗总体治疗效果非常好,大大提高了RB患儿的生存率和保留眼球及有用视力的比率。

Objective To evaluate the therapeutic efficiency of systemic chemotherapy with local therapy on RB.Methods We summarized the data of 42 patients with bilateral intraocular retinoblastoma diagnosed in our hospital from December 2009 through February 2011.22 male and 20 female.The patients were treated with systemic chemotherapy consisted of vincristine,etoposide,and carboplatin（VEC） and local control（laser therapy,cryotherapy）.The clinical characteristics,eye preservation rate and complications of chemotherapy were assessed.Results（1）The median age was 13 months,younger than unilateral RB patients diagnosed at the same time,but without significant statistical difference（P = 0.075）.（2） The most clinical manifestation is leukocoria（29 patients,accounting for69%）.（3） Treatment and prognosis：Of 84 ocular retinoblastomas,3（3.6%） in Group A;9（10.7%）in Group B;10（11.9%）in Group C;40（47.6%）in Group D;22（26.2%）in Group E.21 patients had enucleation.67.8 percent of the eyes are preserved.The preserving eye rate was 50%in group E,62.5%in group D;90%in group C,none in group B and group A.In Group D and E,significant difference（P=0.016） was found between the groups.Among 42 patients 5 were dead（12%）,4 cases got tumor progression,1 case developed intracranial hemorrhage after chemotherapy.Only 3 patients developed transient hearing losses,hearing recovered during follow up.Conclusions No ototoxicity was noticed based on our study.Chemotherapy combined with local ophthalmic therapy is effective for treatment of intraocular retinoblastoma even reducing eye enucleations without significant systemic toxicity.