This study examined the salient clinical and epidemiological character
istics of retinoblastoma (RB) in India, thereby highlighting the probl
ems encountered there. The epidemiological characteristics of 296 pati
ents with RB over 8 years were evaluated using hospital records and po
stal follow-ups. Unilateral disease was sea in 61.8 % of patients. The
overall median age at presentation was 3.5 years (3.5 years for unila
teral 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. Consangu
ineous marriage was sea in 17% and family history of RB was noted in 1
.7% cases. Also, 2% had a history of other malignancy in the family. A
ssociated 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 s
eries. Only 43.6% of patients had disease localized to the globe witho
ut any infiltration/invasion. The majority of cases had advanced-stage
disease at presentation and came from the underprivileged class of so
ciety. Patients with bilateral RB presented much earlier than those wi
th unilateral disease. In patients with unilateral RB, higher age at p
resentation as well as advanced disease may be related to much delay i
n seeking medical attention. In view of the advanced stage at presenta
tion, there also exist a possibility of difference in the biology of t
he tumor sea in these patients.