Clinical Presentation and Survival of Children with Retinoblastoma Treated at the National Eye Centre in Indonesia.
Retinoblastoma is a malignant ocular tumor in children. Patient characteristics differ between low- and middle-income countries and high-income countries. This study analyzes the clinical presentation and survival rates of patients with retinoblastoma at the National Eye Center (NEC) in Indonesia, a middle-income country.
Clinical data of all children diagnosed with retinoblastoma at NEC between 2012 and 2021 were retrospectively analyzed.
In 10 years, 395 patients with retinoblastoma were diagnosed and treated at NEC. The number of diagnoses increased from 2012 to 2019, but declined during the COVID-19 pandemic. Most patients were diagnosed under 5 years of age. The most common sign was leukocoria (77.5%), followed by proptosis (14.0%). Proptosis was even more common in children from remote areas of Indonesia (20.0%). At NEC, 73.4% had unilateral and 26.6% bilateral retinoblastoma. Patients with bilateral retinoblastoma were statistically noticeably younger at diagnosis than unilateral retinoblastoma (unilateral: 27 months; bilateral 21 months; p<0.001). In patients diagnosed between 2017 and 2021, overt extraocular retinoblastoma (IRSS III and IV) was detected in 16.1% while only 5.4% of patients presented with IRSS 0/I at diagnosis. The 3-year overall survival rate for patients diagnosed between 2017 and 2021 was 53.5% (95%-CI: [43.1%, 66.4%]).
In Indonesia extraocular disease at diagnosis is common contributing to low survival rates. To address this, early detection programs, standardized referral pathways, and treatment guidelines have been implemented. These efforts aim to improve survival outcomes and bring them closer in line with those of other middle-income countries.
Clinical data of all children diagnosed with retinoblastoma at NEC between 2012 and 2021 were retrospectively analyzed.
In 10 years, 395 patients with retinoblastoma were diagnosed and treated at NEC. The number of diagnoses increased from 2012 to 2019, but declined during the COVID-19 pandemic. Most patients were diagnosed under 5 years of age. The most common sign was leukocoria (77.5%), followed by proptosis (14.0%). Proptosis was even more common in children from remote areas of Indonesia (20.0%). At NEC, 73.4% had unilateral and 26.6% bilateral retinoblastoma. Patients with bilateral retinoblastoma were statistically noticeably younger at diagnosis than unilateral retinoblastoma (unilateral: 27 months; bilateral 21 months; p<0.001). In patients diagnosed between 2017 and 2021, overt extraocular retinoblastoma (IRSS III and IV) was detected in 16.1% while only 5.4% of patients presented with IRSS 0/I at diagnosis. The 3-year overall survival rate for patients diagnosed between 2017 and 2021 was 53.5% (95%-CI: [43.1%, 66.4%]).
In Indonesia extraocular disease at diagnosis is common contributing to low survival rates. To address this, early detection programs, standardized referral pathways, and treatment guidelines have been implemented. These efforts aim to improve survival outcomes and bring them closer in line with those of other middle-income countries.
Authors
Susanty Susanty, Oktarima Oktarima, Mardianty Mardianty, Maliani Maliani, Kartiwa Kartiwa, Kusumadjati Kusumadjati, Noviyanti Noviyanti, Utami Utami, Leoni Leoni, Maulana Maulana, Fischhuber Fischhuber, Ketteler Ketteler
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