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Updated: Sep 4, 2025
Reconstruct Human Retinoblastoma In Vitro
Published on: October 11, 2022
The Global Retinoblastoma Outcome Study: a prospective, cluster-based analysis of 4064 patients from 149 countries
Insights
Global retinoblastoma survival rates show significant disparities. Children in low-income countries face much lower survival rates for this common eye cancer, highlighting the need for earlier diagnosis and treatment.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Global Health
Background:
- Retinoblastoma is the most prevalent intraocular cancer globally, affecting children worldwide.
- Existing evidence suggests regional variations in retinoblastoma treatment outcomes, but a comprehensive global assessment was lacking.
- This study addresses the need to evaluate global 3-year survival rates and identify factors influencing outcomes in retinoblastoma patients.
Purpose of the Study:
- To report global 3-year survival outcomes for children diagnosed with retinoblastoma.
- To investigate factors, including country income level and tumor stage, associated with retinoblastoma survival.
- To highlight disparities in treatment outcomes and survival rates across different global economic regions.
Main Methods:
- A prospective, cluster-based analysis of 4064 treatment-naive retinoblastoma patients from 149 countries diagnosed in 2017.
- Data collection included primary/additional treatments, follow-up duration, metastasis, globe salvage, and survival outcomes over 3 years.
- Cox regression models were used to analyze time to death and enucleation, identifying independent prognostic factors.
Main Results:
- Survival rates varied dramatically by country income: 99.5% in high-income, 91.2% in upper-middle, 80.3% in lower-middle, and 57.3% in low-income countries.
- Advanced tumor stage (cT4) and older age at diagnosis were associated with worse survival.
- Independent factors for worse survival included residence in low-income countries (HR 16.67) and advanced tumor stage (HR 8.98).
Conclusions:
- Profound inequities exist in retinoblastoma survival, strongly correlated with a country's economic status.
- While essential treatments are widely available, early diagnosis and timely intervention are critical for improving survival in low-income settings.
- Addressing socioeconomic disparities is crucial to reduce mortality from retinoblastoma globally.
Background:
Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival.
Methods:
We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models.
Findings:
The cohort included 4064 children from 149 countries. The median age at diagnosis was 23·2 months (IQR 11·0-36·5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0·8%) of 636 children from high-income countries, 55 (5·4%) of 1027 children from upper-middle-income countries, 342 (19·7%) of 1738 children from lower-middle-income countries, and 196 (42·9%) of 457 children from low-income countries. Enucleation surgery was available for all children and intravenous chemotherapy was available for 4014 (98·8%) of 4064 children. The 3-year survival rate was 99·5% (95% CI 98·8-100·0) for children from high-income countries, 91·2% (89·5-93·0) for children from upper-middle-income countries, 80·3% (78·3-82·3) for children from lower-middle-income countries, and 57·3% (52·1-63·0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16·67; 95% CI 4·76-50·00), cT4 advanced tumour compared to cT1 (8·98; 4·44-18·18), and older age at diagnosis in children up to 3 years (1·38 per year; 1·23-1·56). For children aged 3-7 years, the mortality risk decreased slightly (p=0·0104 for the change in slope).
Interpretation:
This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes.
Funding:
Queen Elizabeth Diamond Jubilee Trust.
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