Late presentation of retinoblastoma in Malaysia

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Abstract
Aims The aims of this study were to review the presenting features, treatment and outcome for Malaysian children with retinoblastoma currently. Subjects and Methods This was a prospective study, conducted at the General Hospital Kuala Lumpur from August 2001until October 2007. Clinical data were collected at presentation and follow‐up. Results One hundred five children were diagnosed to have retinoblastoma. There were 55 males and 50 females, ranging in age from 1 month to 14 years (median 20 months). Seventy‐six children were Malay in ethnic origin (73%), 14 Chinese (13%), 12 Indian (11%), and other races (3%). Sixty‐four children presented with leukocoria (61%), followed by 23 with proptosis (22%), 13 with squint (12%), and 3 with orbital cellulitis (3%). Thirty‐three children (31%) deferred treatment for 6 months or more. Overall, 56 children had extraocular disease (55%), 52 at presentation, 4 later. Seventy‐one children (68%) underwent primary enucleation, 76 received chemotherapy (72%), and 23 radiotherapy (22%). Fifty‐seven children are alive (54%), of whom 3 are blind (5%). Twenty‐seven children were lost to follow‐up (26%) and 21 have died (20%). Conclusion Retinoblastoma in Malaysia is still characterized by predominantly extraocular disease due to late presentation and high rates of abandonment. Pediatr Blood Cancer 2009;52:215–217.