Presentation of Retinoblastoma Patients in Malaysia

Abstract

<b>Background:</b> Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreatedit can cause blindness and even death. The aim of this study is to determine sociodemographic and clinicalfeatures of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia.Materials and <br/><b>Methods</b>: Data for this study were retrieved from the Retinoblastoma Registry of the NationalEye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and HospitalQueen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in theregistry cover demography, clinical presentation, modes of treatment, outcomes and complications. <br/><b>Results</b>:The study group consisted of 119 patients (162 eyes) diagnosed with retinoblastoma between 2004 and 2012.There were 68 male (57.1%) and 51 (42.9%) female. The median age at presentation was 22 months. A majorityof patients were Malays (54.6%), followed by Chinese (18, 5%), Indians (8.4%), and indigenous races (15.9%).Seventy six (63.8%) patients had unilateral involvement whereas 43 patients had bilateral disease (36.1%). It wasfound that most children presented with leukocoria (110 patients), followed by strabismus (19), and protopsis(12). Among the 76 with unilateral involvement (76 eyes), enucleation was performed for a majority (79%).More than half of these patients had extraocular extension. Of the 40 who received chemotherapy, 95% weregiven drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes wereenucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemicchemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not availablein 33 patients. <br/><b>Conclusions</b>: Patients with retinoblastoma in this middle-income Asian setting are presenting atlate stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. Wealso showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initialtreatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in thispart of the world.