Retinoblastoma in Iran: outcomes in terms of patients' survival and globe survival.

BACKGROUND/AIMS: Retinoblastoma is a highly malignant eye tumour in children with different survival rates across the world. The aims of this study are to determine the globe and patient survival in children with retinoblastoma in a major referral centre in Iran.

METHODS: 156 eyes of 105 consecutive patients with retinoblastoma were enrolled from 2001 to 2007. All demographic data, family history, presenting symptoms, duration of symptoms, ocular findings and treatment modalities that were used for the patients were collected. For patient survival, event was defined as death and for globe survival as enucleation.

RESULTS: The mean age at diagnosis was 28.5 months (unilateral 27.4 months; bilateral 30 months). Five patients had a positive family history. Fifty-two per cent of the cases were unilateral, and 48% were bilateral. The most common presenting sign was leucocoria (64.8%) followed by strabismus (28.2%). Enucleation was done primarily for 75.9% of unilateral cases and 34.3% of bilateral cases. Secondary enucleation was necessary in 5.6% and 7.8% of unilateral and bilaterally involved eyes respectively. Sixty-nine (44.2%) of 156 eyes were salvaged by different globe preserving modalities (unilateral 18.5%; bilateral 57.9%). The Kaplan-Meier survival estimate for globe preservation according to International Classification of Retinoblastoma (ICRB) was 100% for group A eyes, 93.5% for group B, 86.7% for group C, 57.1% for group D and 0% for group E eyes. Kaplan-Meier estimates for patients survival were 100% at 1 year, 94.8% at 3 years and 83.1% at 5 years.

CONCLUSION: Progress in methods of treatment, early detection of the disease and prompt referral to specialised centres have led to improved outcomes for patients with retinoblastoma in terms of globe and patients' survival rates even in developing countries.