Retinoblastoma, the Costa Rican Experience

Abstract

Abstract: : Purpose: To report the epidemiological, treatment and survival characteristics of patients with retinoblastoma in Costa Rica. Methods: Retrospective, transversal study of 48 eyes from 35 consecutive patients diagnosed with retinoblastoma and managed at the Hospital Nacional de Niños from January 1997 to March 2003. Charts were analized for epidemiological, clinical, treatment and survival related data. Results: Four eyes were incorrectly diagnosed as retinoblastoma and enucleated. The remaining 44 eyes from 31 patients are the subject of this report. Incidence, laterality, family history and presenting symptoms occur in a similar frequency to that reported in developed countries. The mean age of diagnosis of the 13 bilateral cases was 15 months compared to 25 months in the 18 unilateral cases. At the time of diagnosis only one eye had extraocular extension. Enucleation was the preferred method of treatment. At the end of the study period, 30 eyes had undergone enucleation. Chemotherapy was performed either primarily or at some point of their treatment in 26 eyes. During follow–up, two thirds of patients had to undergo additional treatment. 7 patients developed extraocular extension into the bone marrow and/or the CNS. 10.7% of enucleated specimens had tumor involvement up to the edge of the cut optic nerve. A total of 6 patients died making the overall survival rate only 80.6%. Conclusions: Although epidemiological characteristics of retinoblastoma are similar in Costa Rica to those of developed countries, treatment outcomes are poorer. Standardized diagnostic and treatment protocols that include chemoreduction need to be implemented in Costa Rica if we want to improve survival and ocular salvage rates.

Keywords: retinoblastoma • oncology