Clinical profile, management, and outcome of retinoblastoma in singapore.

PURPOSE: To analyze the clinical manifestations and treatment outcomes for patients with retinoblastoma in Singapore from 1997 to 2010. METHODS: Medical records of 51 patients (67 eyes) diagnosed as having retinoblastoma were analyzed. Data on laterality, genetics, presentation, disease severity, treatment, and prognosis were collected. RESULTS: The mean age of presentation was 25.7 ± 19.9 months. Sixteen (31.4%) of the patients had bilateral disease, of whom 2 had an associated pineal tumor. Leukocoria was the most common sign. Two had metastasis at diagnosis. Only 3 patients (5.9%) had a family history. Using the International Intraocular Retinoblastoma Classification, 6.0% were Group A, 6.0% were Group B, 3.0% were Group C, 38.8% were Group D, and 49.2% were Group E. Chemotherapy and focal therapy were administered for 4 of 35 (12.9%) patients with unilateral retinoblastoma (50% had successful globe preservation) and 13 of 16 (81.3%) patients with bilateral retinoblastoma (42.3% had successful globe preservation). Overall, globe preservation was achieved in 100% of Groups A, B, and C, and 23.1% of Group D cases. The 5-year survival rate overall, for unilateral retinoblastoma, and for bilateral retinoblastoma was 91%, 97%, and 76% respectively. CONCLUSION: The overall 5-year survival rate is comparable to international data in most developed countries. However, most patients presented with advanced disease, making the rate of globe preservation lower than in some developed countries. Better education of the public and healthcare professionals may increase awareness and enable early detection of the disease.