ABSTRACT
Introduction: Retinoblastoma is a very rare disease. There were 30 cases of retinoblastoma diagnosed and treated at National University Hospital (NUH). Materials and Methods: A retrospective chart review was performed on the medical records of 30 patients who were diagnosed with retinoblastoma between 1995 and 2008 at the Department of Paediatrics, National University Hospital, Singapore. Results: The median age at diagnosis was 1.6 years (range, 0-5.9) with a median follow-up of 1.8 years (range, 0.1 to 11.6). The median time from presenting signs to the time of diagnosis was 5.2 months (range, 0-25.2). Common presenting signs of retinoblastoma were identified; the most common of which were leukocoria (50.0%), squinting (13.3%), poor vision (10.0%), strabismus (6.6%) and unknown (33.3%). Of the 30 patients, 10 were from Singapore whilst the other 20 patients were from the surrounding countries. Twelve patients had bilateral disease at the time of diagnosis, while 18 had unilateral disease. Staging information was available in 27 patients. Enucleation was performed in 25 of 30 patients. Radiation therapy was given in 3 patients in 1995 (bilateral disease), 2001 (bilateral disease) and 2003 (unilateral disease). At the time of analysis, 19 patients were alive with no evidence of disease. Overall 5-year survival for the cohort was 88.1% [95% confidence interval (CI), 88.0-100] and event-free survival for the whole cohort was 74.2% (95% CI, 55.8-92.6). Conclusion: In our limited experience, the importance of collaboration and standardisation of the staging system, raising awareness and education of primary healthcare providers and parents are strongly stressed.
Retinoblastoma is the most common primary intraocular malignancy in infants and children.1 Worldwide, the incidence is 1 in 18,000 to 34,000 live births with an estimation of 5000 to 8000 new cases.2,3 The Singapore Childhood Cancer Registry (SCCR), a hospital based cancer registry that includes all children less than 15 years of age, which was initiated by the Paediatric Oncology Group-Singapore (POGS), reported their fi rst monograph in 2007. They noted 21 cases of retinoblastoma diagnosed at National University Hospital (NUH) between 1997 and 2005.4 However, there is limited information regarding the presentation of symptoms, treatment and outcome of retinoblastoma in children in Singapore.
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