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一项基于人群的阿根廷儿童视网膜母细胞瘤发病率及生存率研究。

A population-based study of retinoblastoma incidence and survival in Argentine children.

作者信息

Moreno Florencia, Sinaki Banafsheh, Fandiño Adriana, Dussel Verónica, Orellana Liliana, Chantada Guillermo

机构信息

Registro Onco-pediatrico Hospitalario Argentino (ROHA, Hospital-based Pediatric Cancer Registry from Argentina), Instituto Nacional del Cáncer, Buenos Aires, Argentina.

出版信息

Pediatr Blood Cancer. 2014 Sep;61(9):1610-5. doi: 10.1002/pbc.25048. Epub 2014 Apr 11.

DOI:10.1002/pbc.25048
PMID:24729462
Abstract

BACKGROUND

An increased incidence of retinoblastoma in some developing countries has been reported but no conclusive data are available from population-based studies at national level.

PURPOSE

To report the incidence and survival of retinoblastoma in Argentina from the National Pediatric Cancer Registry (ROHA) and the influence of socio-economical indicators on outcome.

PROCEDURE

Cases reported to the ROHA (2000-2009) were analyzed. Incidence rates were calculated using National Vital Statistics and survival was estimated. The extended human development index (EHDI) was used as a socio-economical indicator.

RESULTS

With 438 patients reported, an incidence of 5.0 cases per million children 0-14 years old (95% CI 3.5-6.4) was calculated. Median age at diagnosis was significantly higher for children from provinces with lower EHDI; (24 vs. 35 months for unilateral, (P = 0.003) and 9 versus 11.5 months for bilateral retinoblastoma (P = 0.027). The 3-year probability of survival was 0.87 and 0.94 for unilateral and bilateral retinoblastoma, respectively. Residents in provinces with higher EHDI had a better 3-year survival (0.93 vs. 0.77 for lower EHDI, P < 0.0001). Probability of survival was higher for patients treated at tertiary level institutions (P = 0.0015). The combination of low EHDI residence province with no treatment at a tertiary institution was associated with the worst survival outcome. For both, unilateral and bilateral disease, children who died were in average diagnosed at older age.

CONCLUSIONS

The incidence of retinoblastoma in Argentina is comparable to that of developed countries. Retinoblastoma is diagnosed later and survival is lower in the less developed areas of the country.

摘要

背景

据报道,一些发展中国家视网膜母细胞瘤的发病率有所上升,但尚无来自国家级基于人群研究的确切数据。

目的

通过国家儿科癌症登记处(ROHA)报告阿根廷视网膜母细胞瘤的发病率和生存率,以及社会经济指标对预后的影响。

方法

分析向ROHA报告的病例(2000 - 2009年)。使用国家生命统计数据计算发病率并估计生存率。扩展人类发展指数(EHDI)用作社会经济指标。

结果

报告了438例患者,计算出0 - 14岁儿童的发病率为每百万儿童5.0例(95%可信区间3.5 - 6.4)。EHDI较低省份儿童的诊断中位年龄显著更高;单侧视网膜母细胞瘤为24个月对35个月(P = 0.003),双侧视网膜母细胞瘤为9个月对11.5个月(P = 0.027)。单侧和双侧视网膜母细胞瘤的3年生存率分别为0.87和0.94。EHDI较高省份的居民3年生存率更好(EHDI较低者为0.93对0.77,P < 0.0001)。在三级医疗机构接受治疗的患者生存率更高(P = 0.0015)。EHDI低的居住省份且未在三级机构接受治疗与最差的生存结果相关。对于单侧和双侧疾病,死亡儿童的平均诊断年龄较大。

结论

阿根廷视网膜母细胞瘤的发病率与发达国家相当。在该国较不发达地区,视网膜母细胞瘤的诊断较晚且生存率较低。

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