Abstract
Purpose: :
Advanced intraocular tumors and metastatic disease at diagnosis are a major concern in developing countries. We aimed to report the presentation status, clinical characteristics and outcome of retinoblastoma patients in a government health institution.
Methods: :
Retrospective chart review of 210 patients who presented to the National Institute of Cancer (INCA), Rio de Janeiro, Brazil from 1991 to 2006, with newly diagnosed retinoblastoma. All patients with intraocular disease were staged using the International Classification of Retinoblastoma and for the extra-ocular disease the Chantada international retinoblastoma staging system.
Results: :
Unilateral disease was present in 143 (69%) of cases. Mean age at diagnosis of intraocular disease was 32 months old for unilateral and 18 months old for bilateral cases. For extra-ocular disease, mean age at diagnosis was 39 months old for unilateral and 21 months old for bilateral cases. Extra-ocular disease was present in 38,2% of the patients. The median time between presenting signs to diagnosis of patients with intra-ocular and extra-ocular disease was 6 months (range: 0,25_45 months) and 11 months (range 0,5 - 34 months), respectively, U=2,36 (p=0,018). Higher mortality rate was associated with longer time to diagnosis of extra-ocular disease (51,2%) compared to intra-ocular disease (7,8%) [RR=6,6 (4.04-10,78)][x2=54,40 p=0.001].
Conclusions: :
Advanced cases with delayed diagnosis are related to higher mortality in this series of patients. Given the elevated number of patients with advanced intraocular stages and extra-ocular disease, it is mandatory to develop a national early detection program.
Keywords: retinoblastoma • tumors • detection