Retinoblastoma: Problems and Perspectives from 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.

Presentation of retinoblastoma at a paediatric eye clinic in Ghana

“…24 Sahu et al in their retrospective study of 296 affected patients with retinoblastoma, over an 8 years period, in Mumbai, India, also found a predominance of advanced-stage disease with 74.5% presenting with Reese-Ellsworth group IV and V disease. 20 These findings are supported by our study.…”

“…As survival in patients with retinoblastoma is inversely related to the stage of the disease at presentation, [20][21][22][23][24] we carried out this study to determine the stages of retinoblastoma in our patients at presentation and their relation to outcome. This will serve as baseline data for further studies and help plan interventions for improvement in survival.…”

Presentation of Retinoblastoma Patients in Malaysia

“…This study showed that the majority of patients (64%) presented with unilateral retinoblastoma and the remaining (36%) are bilateral. This figure is similar with findings from several Asian countries (Sahul et al, 1998;Patikulsila et al, 2001). In this study, there was not much difference in the number of retinoblastoma between male and female; male to female ratio of 1.3:1.…”

Metastatic and Nonmetastatic Models of Retinoblastoma

“…Only animals in the Y79 tumor group had tumors that appeared to mimic the aggressive, rapidly growing, metastatic retinoblastomas only occasionally observed in North America and Europe but more commonly seen in Mexico, Central and South America, Saudi Arabia, and India. 29 The WERI-Rb animal model behaved like patients with nonmetastatic retinoblastoma more commonly seen in Europe and North America.…”