244例儿童视网膜母细胞瘤临床特点

目的 分析和探讨儿童视网膜母细胞瘤(Rb)的临床特点.设计 回顾性病例系列.研究对象 北京同仁医院2009年1月至12月收治的244例(312眼)儿童视网膜母细胞瘤患者的病历资料.方法 对上述患儿临床病历资料进行回顾,分析患儿的发病年龄、首诊症状、家族史,肿瘤的临床分期、治疗方法、随诊结果及疗效等.主要指标 发病年龄、...

Study on the clinical characteristics of 244 retinoblastoma cases

Objective To present the clinical characteristics of retinoblastoma （Rb） cases. Design Retrospective case series. Participants The clinical information of 244 Rb cases from Jan. 2009 to Dec. 2009. Methods The age at initial diagnosis, symptoms at presenting, family history, clinical stage, treatment scenario and therapeutic effect were retrospectively analyzed. Main Outcome Measures The age at initial diagnosis, sex, clinical stage and therapeutic effect. Results （1） The mean age at initial diagnosis was 1.97±1.42 years （range 0.08-8 years）, medium 1.75 years. 83.19% （203/244） of the children was younger than 3 years old. （2） 137 of the children were male, 107 of them were female, the difference was not significant （x2=0.05,P=0.240）. （3） Leucocoria was the most common symptom at presenting, which happened in about 67.62% of cases （165/244）. （4） In all the 244 cases, 8 cases （3.27%） showed the positive family history. Among them, 5 cases were binocular Rb, and the other 3 cases were monocular Rb. （5） Rb stage： expect for 10 patients who had been performed enucleation of eyeball before coming to Beijing Tongren Hospital, according to IRC stage, 14.56% （44/302） of the cases was A-C stage, 41.39%（125/302） was D stage, and 44.05%（125/302） was E stage. （6） Enucleation of eyeball was performed on 50 patients. Radiotherapy, chemotherapy combined with local treatment were performed on 104 patients. The combined treatment plus enucleation were performed on 90 cases. （7） The patients were followed up for 12 to 24 months. The survival rate was 97.37% （222/228）, and mortality rate was 2.63% （6/228）, except for 16 cases losing to follow-up or giving up the treatment. Conclusion Rb is early onset. Leucocoria is the most common symptom. Most of the cases are in the end-stage. And half of them were performed enucleation of eyeball.