Epidemiological profile of retinoblastoma in North India: Implications for primary care and family physicians.

Abstract

Background:Retinoblastoma is the most common primary intraocular malignancy among children. Despite being curable in early stages, majority of the cases in India present in late stages, when outcomes are very poor.Objectives:The aim of this study was to assess the epidemiological profile, clinical characteristics, and treatment practices among retinoblastoma patients in north India.Materials and Methods:Data on all patients with retinoblastoma, over a 10-year-time period from 2009 to 2018, who were treated in a tertiary care hospital in north India, were assessed. Data were analyzed to describe the demographic characteristics, clinical features in terms of stage at presentation, and management practices in terms of diagnostic investigations and treatment. The statistical significance for difference in percentages was assessed using Fischer's exact test at a 5% significance level.Results:A total of 25 retinoblastoma patients were enlisted, of whom one was excluded as it was adult onset retinoblastoma. The median age at presentation was 3 years, with a male to female ratio of 1:1.4. Bilateral presentation was seen in 16.6% cases. Majority (66.6%) of the patients underwent magnetic resonance imaging of brain and orbit as a part of the diagnostic workup. Intraocular disease was seen in 58.3% patients, whereas 41.6% patients had extraocular disease. Local therapy with vision preservation could be used only in 8.3% patients, whereas 87.5% patients were referred for enucleation. Chemotherapy with combination of vincristine, etoposide, and carboplatin was used extensively both, in neoadjuvant setting (83.3%) and in the adjuvant setting.Conclusion:Despite availability of treatment for eye preservation, its utility is limited due to the advanced stage at presentation. Awareness about the disease and its symptoms for early diagnosis, especially with the Mid-Level Health Provider at Health and Wellness Centers, is likely to improve early reporting and treatment and meeting the Vision 2020 goals.