Retinoblastoma Outcomes in the Americas: a prospective analysis of 491 children with retinoblastoma from 23 American countries
ABSTRACT
Purpose:
Globally, disparities exist in retinoblastoma treatment outcomes between high- and low-income countries, but independent analysis of American countries is lacking. We report outcomes of American retinoblastoma patients and explore factors associated with survival and globe salvage.
Design:
Subanalysis of prospective cohort study data.
Methods:
Multicenter analysis at 57 American treatment centers in 23 countries of varying economic levels (low income=LIC, lower middle=LMIC, upper-middle=UMIC, high=HIC) of 491 treatment-naïve retinoblastoma patients diagnosed in 2017 and followed through 2020. Survival and globe salvage rates analyzed with Kaplan-Meier analysis and Cox proportional hazard models.
Results:
Of patients, 8 (1.6%), 58 (11.8%), 235 (47.9%) and 190 (38.7%) were from LIC, LMIC, UMIC and HIC, respectively. Threeyear survival rates in LICs were 60.0% (95% CI, 12.6-88.2) compared to 99.2% (94.6-99.9) in HICs. Death was less likely in patients older than four years (vs. four or younger, HR=0.45 [95% CI, 0.27 – 0.78], P=0.048). Patients with more advanced tumors (e.g., cT3 vs. cT1, HR= 4.65×109 [95% CI, 1.25×109 – 1.72×1010], P<0.001) and females (vs. males, HR=1.98 [1.27-3.10], P=0.04) were more likely to die. Three-year globe salvage rates were 13.3% (95% CI, 5.1-25.6) in LMICs and 46.2% (38.8-53.3) in HICs. At three years, 70.1% of cT1 eyes (95% CI, 54.5-81.2) versus 8.9% of cT3 eyes (5.5- 13.3) were salvaged. Advanced tumor stage was associated with higher enucleation risk (e.g., cT3 vs. cT1, SHR=4.98 [95% CI, 2.36-10.5), P<0.001).
Conclusions:
Disparities exist in survival and globe salvage in American countries based on economic level and tumor stage demonstrating a need for childhood cancer programs.
