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.
Highlights
Retinoblastoma (RB) is the most common intraocular malignancy in childhood and accounts for about 2% of all childhood cancers [1]
The purpose of our study was to analyse the survival outcomes of adjuvant therapy based on histopathological risk stratifica-tion in patients who underwent upfront enucleation for unilateral RB with advanced disease
Proptosis and vision loss were the usual presenting clinical signs associated with advanced group disease D and E (p value 0.002)
Summary
Retinoblastoma (RB) is the most common intraocular malignancy in childhood and accounts for about 2% of all childhood cancers [1]. RB occurs due to germline or somatic. Loss of function of RB 1 gene on chromosome 13q results in the germline (hereditary) RB, which commonly presents as bilateral eye involvement in early age group. Somatic RB (non-heritable RB) results from loss of function of RB1 gene in the retina; patients will usually have unilateral, unifocal disease and present at a later age compared to the heritable type [3]
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