大龄儿童视网膜母细胞瘤发病特点及预后观察

The clinical characteristics and prognosis of 92 cases of delayed occurrence of retinoblastoma

目的总结5岁及以上儿童视网膜母细胞瘤（RB）的临床发病特点及疗效。方法系列病例研究。对2005年9月至2012年5月首都医科大学附属北京同仁医院儿科收治的发病年龄〉／5岁，且经临床诊断和（或）病理确诊为RB的92例（107只眼）患儿的发病特点、初诊症状、临床缓解情况及随访结果进行回顾性分析。生存函数采用Kaplan—Meier曲线分析法。结果（1）92例患儿的发病中位年龄为6．25岁（5～18岁）。男性61例（66．30％），女性31例（33．69％）。患儿年龄分布主要集中在5．00～8．33岁，其中第25百分位年龄为5．77岁，第50百分位年龄为5．83岁，第75百分位年龄为7．85岁。（2）RB以单眼发病为主者77例（83．69％），双眼发病者15例（16．31％）。（3）患儿发病初诊时常见症状为白瞳和黄白色反光者占55．43％（51／92），视力下降、眼球充血和畏光者占23．91％（22／92），斜视、眼球突出、青光眼和体检发现者占20．64％（19／92）；92例患儿均无相关家族史。（4）随访至2013年2月，107只患眼中行眼球摘除治疗76只，总眼球摘除率为71．02％。（5）随访至2013年2月，随访中位时间39个月（9～78个月），失访1例，死亡6例，总生存率为93．46％（85／92）。Kaplan—Meier生存曲线分析结果显示，5年预计平均总生存时间估计值为（72．61±1．89）个月，95％可信区间为68．9—76．3个月。结论5岁及以上RB多为单眼发病，以白瞳征为主要发病症状，经综合治疗总生存率较高。

Objective To retrospectively summarize the clinical features, diagnosis and treatment of retinoblastoma（RB） patients in the children aged 5 years or above. Methods This was a case-series study. Total of 92 patients （107 eyes ） with RB aged 5 years or above in Department of Pediatric, Beijing Tongren Hospital from September 2005 to May 2010 were included in the study. The diagnosis of RB was established using clinical examination and histopatho]ogy analysis. Statistical analysis in the characteristics of disease and initial symptoms was performed in 92 cases. Kaplan-Meier curve was employed to analyze the survival function. Results （ 1 ） The medium age of the patients was 6. 25-year. Male accounted for 66. 30% （61/92） and female was 33.69% （31/92）. According to the results of statistical scatter diagram,the age of the disease onset was ranged from 5.00 to 8.33 years. Percentile for age 5.77,5.83 and 7. 85 years were the 25th ,50th ,75th respectively. （2）In 92 cases, 83.69% （77/92） of the patients were unilateral RB and 16. 31% （15/92） were bilateral RB. （3）Leucocoria was the most ＂common manifestation in the diagnosis early RB, accounting for 55.43% （51/92） . Secondary symptoms were blurred vision, eye redness and photophobia,accounting for 23.91% （22/92）. Other symptoms included strabismus,proptosis,glaueoma and the abnormal in fundus examination ,accounting for 20. 64% （19/92）. No family history of RB was found in all of cases studied. （4）Among 107 eyes with RB,76 eyes were enucleated up to February 2013. The rate of enucleation was 71.02%. （5）By February 2013, the average following-up time was 39 months, during the time 1 case was lost, 6 cases were dead, the overall survival rate was 93.46% （85/92）. The result of Kaplan-Meier curve showed that the estimates of 5-year expected average total survival time was （72. 61 ± 1.89） months （95% confidenee interval was 68.9-76. 3 months）. Conclusions Unilateral RB are the most common cases in the age of 5 years or above,and the most common symptom is Leucocoria. Overall survival rate of older RB patient is high with comprehensive treatment.