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Retinoblastoma: problems and perspectives from India
S Sahu1, S D Banavali, S K Pai
1Department of Medical Oncology, Tata Memorial Hospital, Mumbai, India.
Insights
This study on retinoblastoma (RB) in India found most patients present with advanced disease, often from underprivileged backgrounds. Early detection is crucial, especially for unilateral cases, to improve outcomes for this childhood eye cancer.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Epidemiology
Background:
- Retinoblastoma (RB) is the most common primary intraocular malignancy in children.
- Understanding the clinical and epidemiological profile of RB is crucial for early diagnosis and effective management, particularly in diverse healthcare settings like India.
Purpose of the Study:
- To investigate the clinical and epidemiological characteristics of retinoblastoma in Indian patients.
- To identify challenges and disparities in the presentation and management of retinoblastoma in India.
Main Methods:
- Retrospective analysis of 296 retinoblastoma cases over 8 years.
- Data collection through hospital records and postal follow-ups.
- Evaluation of patient demographics, disease laterality, age at presentation, family history, and disease stage.
Main Results:
- Unilateral RB occurred in 61.8% of patients; median age at presentation was 3.5 years (1.0 year for bilateral).
- 74.5% presented with advanced disease (Reese-Ellsworth groups IV and V), with only 43.6% localized to the globe.
- Patients from underprivileged backgrounds and those with unilateral disease presented later with more advanced stages.
Conclusions:
- A significant proportion of Indian retinoblastoma patients present with advanced disease, often linked to delayed medical attention and socioeconomic factors.
- Bilateral RB cases present earlier than unilateral ones, suggesting potential differences in tumor biology or awareness.
- Addressing socioeconomic barriers and promoting early detection are vital for improving retinoblastoma outcomes in India.
Abstract:
This study examined the salient clinical and epidemiological characteristics of retinoblastoma (RB) in India, thereby highlighting the problems encountered there. The epidemiological characteristics of 296 patients with RB over 8 years were evaluated using hospital records and postal follow-ups. Unilateral disease was seen in 61.8% of patients. The overall median age at presentation was 3.5 years (3.5 years for unilateral RB and 1.0 years for bilateral RB). The male/female ratio was 1.4:1. The median duration of symptomatic disease was 8 months. Consanguineous marriage was seen in 17% and family history of RB was noted in 1.7% cases. Also, 2% had a history of other malignancy in the family. Associated congenital malformation was seen in 10.5% of cases. A second malignancy was seen in 0.67% of cases at a mean duration of 4.5 years after completion of therapy. A predominance of advanced-stage disease (74.5% had Reese-Ellsworth group IV and V disease) was seen in our series. Only 43.6% of patients had disease localized to the globe without any infiltration/invasion. The majority of cases had advanced-stage disease at presentation and came from the underprivileged class of society. Patients with bilateral RB presented much earlier than those with unilateral disease. In patients with unilateral RB, higher age at presentation as well as advanced disease may be related to much delay in seeking medical attention. In view of the advanced stage at presentation, there also exist a possibility of difference in the biology of the tumor seen in these patients.