Related Experiment Video
Updated: Oct 9, 2026
Reconstruct Human Retinoblastoma In Vitro
Published on: October 11, 2022
Survival outcomes of unilateral retinoblastoma based on pathological risk stratification-experience at a tertiary
Najma Shaheen1, Naila Inayat1, Sehar Bashir2
1Department of Pediatric Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, 7A, Johar Town, Lahore 54782, Pakistan.
Insights
Adjuvant therapy after enucleation for unilateral retinoblastoma (RB) in advanced disease is crucial. High-risk histopathological features correlate with disease progression, emphasizing the need for tailored treatment strategies in childhood intraocular malignancy.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Pathology
Background:
- Retinoblastoma (RB) is the most common childhood intraocular malignancy.
- Upfront enucleation for unilateral RB is common in low-middle income countries due to resource limitations, despite globe-preserving alternatives in high-income nations.
- Adjuvant therapy post-enucleation, guided by histopathology, is vital for reducing metastasis risk in advanced RB.
Purpose of the Study:
- To analyze survival outcomes of adjuvant therapy based on histopathological risk stratification.
- To evaluate treatment effectiveness in patients with unilateral RB and advanced disease who underwent upfront enucleation.
Main Methods:
- Retrospective study of 113 patients (3 months–16 years) with unilateral RB undergoing upfront enucleation (July 2009–January 2019).
- Analysis of histopathological risk features, clinical presentation (leukocoria), and disease staging (Groups D and E).
- Assessment of disease progression, relapse, overall survival, and event-free survival (EFS).
Main Results:
- Majority of patients presented with advanced disease (Group D: 62.8%, Group E: 32.7%).
- High-risk histopathological features were identified in 25.7% of patients, intermediate risk in 47.8%.
- Disease progression and relapse were more frequent in patients with high-risk histopathological features. The 4-year overall survival and EFS were 74% and 71%, respectively.
Conclusions:
- Histopathological risk stratification is essential for tailoring adjuvant therapy in unilateral retinoblastoma post-enucleation.
- Adjuvant chemotherapy in patients with high-risk features is critical for improving survival outcomes.
- Increased nationwide awareness of early RB symptoms is necessary for timely detection and reduced mortality.
Abstract:
Retinoblastoma (RB) is the most common childhood intraocular malignancy. In high-income countries over the past decade, upfront enucleation for unilateral RB is least favoured due to other alternatives that can help in globe preservation, but in low-middle income countries it is still the preferred option due to lack of resources and expertise. The treatment of RB after enucleation is tailored based on the histopathological risk features, as adjuvant chemotherapy with high-risk features reduces the risk of metastasis. The aim of our study was to analyse the survival outcomes of adjuvant therapy based on histopathological risk stratification in patients who underwent upfront enucleation for unilateral RB with advanced disease. A retrospective study was carried out at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Pakistan. A total 113 patients (aged 3 months till 16 years) diagnosed with unilateral RB who had upfront enucleation from July 2009 till January 2019 were included in this study. The mean age of diagnosis was 37.4 months (±24.5) and male-to-female ratio of 1.3:1. The most common clinical presentation was leukocoria (74.3%). Patients who underwent enucleation had advanced disease; group D present in 62.8% followed by group E (32.7%). Histopathology revealed high-risk features in 29 patients (25.7%) and intermediate risk in 54 patients (47.8%). Disease progression and relapse was seen in patients with high-risk histopathological features. The 4-year over-all survival and EFS observed for this cohort was 74% and 71%. Awareness about the early symptoms among the general population and health care personnel at a nationwide level is needed to facilitate early detection and lessen disease related morbidity and mortality.
Related Concept Videos
01:20The Retinoblastoma Gene
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
01:21Cancer Survival Analysis