CURBES: The Cuban Retinoblastoma Epidemiological Study

Author: Ernesto Alemañy-Rubio (Cuba)

Co-authors: Liudmira Gonzalez Rodriguez, Ken Kitayama, Alejandro Ochoa III, David C. Gritz

Purpose

To describe the epidemiology of retinoblastoma (Rb) in Cuba from 2010 to 2016.

Setting/Venue

SETTING: Cuban population: Because Cuba represents a well-defined and enumerated population, it is a unique setting in which to study the epidemiology of disease. VENEU: Cuban Institute of Ophthalmology. Vitreo-Retina Department. Havana. Cuba.

Methods

Medical records of all retinoblastoma cases for the study period from 2010 to 2016 were reviewed utilizing the centralized Cuban Institute of Ophthalmology database managed by the country’s main retinoblastoma specialist. Utilizing medical records data, de-identified demographic information was abstracted. Disease characteristics was also collected. Inclusion criteria consisted of having been diagnosed with retinoblastoma during the study period (2010 to 2016) with medical records data that were available for abstraction. Participant data were excluded if medical records are not available. For the calculation of incidence rates, the number of live births per year was obtained from annual demographic data published by National Office of Statistics (Oficina Nacional de Estadísticas, ONE, for its acronym in spanish). Incidence rates and associated 95% confidence intervals (CI) were calculated using Poisson (log-linear) regression. Five-year overall survival rates were calculated for cases diagnosed during 2010 through 2012.

Results

There were a total of 71 cases of retinoblastoma during the study period (2010 to 2016). The estimated incidence rate of retinoblastoma during the study period was 1.56 cases per 20,000 live births (95% CI: 1.31 to 1.85). The median age at diagnosis was 1.21 years. Just over half of retinoblastoma cases self-reported white race (52.11%). Approximately half of retinoblastoma cases were male (50.70%) and about 59.15% of retinoblastoma cases were unilateral. Thirty-four percent of retinoblastoma eyes had exophytic tumors and 60% of diseased eyes were graded as class D or higher according to the International Classification of Retinoblastoma guidelines. The 5-year overall survival rate was 93.3% (2 deaths by 12/31/2017 in the 30 cases diagnosed during 2010 through 2012).

Conlusions

The incidence rate of Rb in Cuba has remained stable throughout the study period (1.56 cases/20,000 live births, 95% CI: 1.31 to 1.85). This is similar to worldwide estimates (~1/20,000 live births). Despite limited resources, CIO has achieved 5-year survival estimates similar to the US. Five-year survival in Cuba: 93.3%; five-year survival in US: 95.2%.

Financial Disclosure

No financial disclosure

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