Presentation of Retinoblastoma Patients in Malaysia

Abstract

Background: Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreated it can cause blindness and even death. The aim of this study is to determine sociodemographic and clinical features of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia. Materials and Methods: Data for this study were retrieved from the Retinoblastoma Registry of the National Eye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and Hospital Queen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in the registry cover demography, clinical presentation, modes of treatment, outcomes and complications. Results: 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 majority of 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 was found 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% were given drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes were enucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemic chemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4 dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not available in 33 patients. Conclusions: Patients with retinoblastoma in this middle-income Asian setting are presenting at late stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. We also showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initial treatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in this part of the world.

Survival and outcome of retinoblastoma treated by neo‐adjuvant chemotherapy in India

“…The proportion of cases with the extraocular disease in our study was 41%, which was significantly higher than the developed world (less than 5%) . This is comparable to the reports from developing (Taiwan, Malaysia) and underdeveloped regions (Ghana) of the world which ranges from 26% to 50% . Major reasons for late presentation with advanced disease include lack of awareness, inadequate healthcare facilities, unreliable referral system, delay in seeking health care by the guardians, and poor compliance to treatment because of fear of morbidity and treatment failure …”

“…This was higher when compared with other studies from Asia, which report the average/median age at diagnosis from 22 months to 29 months. However, this is better when compared with reports from the African continent where the median age at diagnosis was 36 months (Ghana) and 37.5 months (Kenya) …”

Clinical Features and Long-term Prognosis of Retinoblastoma according to Age at Diagnosis

“…To our knowledge, the present analysis is the first to show that Korean retinoblastoma patients diagnosed at less than 1 year of age showed a tendency to be classified in a lower IIRC stage than did those diagnosed at an older age and that the age at diagnosis is significantly positively correlated with IIRC stage. In a similar context, a few recent studies reported in other parts of Asia showed that a higher proportion of patients with bilateral retinoblastoma rather than unilateral retinoblastoma were diagnosed at an earlier IIRC stage [12,13]. As in our study, these studies also showed that patients diagnosed at an age younger than 1 year were more likely to have bilateral retinoblastoma rather than unilateral retinoblastoma [12,13].…”

“…In a similar context, a few recent studies reported in other parts of Asia showed that a higher proportion of patients with bilateral retinoblastoma rather than unilateral retinoblastoma were diagnosed at an earlier IIRC stage [12,13]. As in our study, these studies also showed that patients diagnosed at an age younger than 1 year were more likely to have bilateral retinoblastoma rather than unilateral retinoblastoma [12,13].…”

Retinoblastoma: An overview of modern management

“…Proptosis, which is a late sign of RB, is still common in developing countries compared with developed countries (Dimaras et al, 2012). In addition, glaucoma and hyphema may mimic RB (Subramaniam et al, 2014). Other presentations can be as a fungating and necrotic orbital mass, buophthalmos with an enlarged and cloudy cornea or with a flat anterior chamber (Essuman et al, 2010).…”