Twenty-Year Collaboration Between North American and South American Retinoblastoma Programs (original) (raw)
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Retinoblastoma : Recognise the Disease early and save a child's life
Retinoblastoma is the most common malignant intraocular tumor of childhood, with an incidence of 1 in 16,000 to 18,000 live births. The most common symptoms of retinoblastoma are leukocoria and strabismus. The diagnosis of retinoblastoma is delayed in the developing countries when compared to that of developed countries. The management of retinoblastoma has dramatically changed over the years from previous radiotherapy methods to current chemotherapy strategies. The treatment of choice in less advanced cases is chemotherapy and in more advanced cases is enucleation. With the improved treatment strategies, patient survival, globe salvage, and vision salvage in patients with retinoblastoma has drastically improved over the years. The aim of this article is to discuss the presenting features, treatment strategies, and prognosis of retinoblastoma.
Lancet, 2022
Background Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival. Methods We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models. Findings The cohort included 4064 children from 149 countries. The median age at diagnosis was 23•2 months (IQR 11•0-36•5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0•8%) of 636 children from high-income countries, 55 (5•4%) of 1027 children from upper-middle-income countries, 342 (19•7%) of 1738 children from lower-middle-income countries, and 196 (42•9%) of 457 children from low-income countries. Enucleation surgery was available for all children and intravenous chemotherapy was available for 4014 (98•8%) of 4064 children. The 3-year survival rate was 99•5% (95% CI 98•8-100•0) for children from highincome countries, 91•2% (89•5-93•0) for children from upper-middle-income countries, 80•3% (78•3-82•3) for children from lower-middle-income countries, and 57•3% (52•1-63•0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16•67; 95% CI 4•76-50•00), cT4 advanced tumour compared to cT1 (8•98; 4•44-18•18), and older age at diagnosis in children up to 3 years (1•38 per year; 1•23-1•56). For children aged 3-7 years, the mortality risk decreased slightly (p=0•0104 for the change in slope). Interpretation This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes. Funding Queen Elizabeth Diamond Jubilee Trust.
“Retinoblastoma survival disparity”: The expanding horizon in developing countries
Saudi Journal of Ophthalmology, 2012
The patients' survival for the most common intraocular tumor in children, retinoblastoma, has a wide spectrum among the world countries. This study was conducted to provide an overview of the retinoblastoma survival disparity worldwide by discussing the trends of patients' survival, as well as recent advances in the management of retinoblastoma. The design of this study was literature review and commentary. Selected articles from PubMed (except one) including both developing and developed countries regarding the patients' survival in retinoblastoma were considered and reviewed critically. An analysis of 47 articles was performed. In conclusion, in spite of an obvious contrast of retinoblastoma survival disparity in the world, the expanding horizons in developing countries are promising and continuing on all fronts and results are hope-inspiring.
Ophthalmology Retina, 2020
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Outcome of extraocular retinoblastoma in a resource limited center from low middle income country
Pediatric hematology and oncology, 2018
Retinoblastoma (RB) is the most common ocular malignancy in children, and is managed by multimodal treatment. There is a paucity of data regarding the clinical profile and outcome of children with extraocular retinoblastoma from Low Middle Income Countries (LMIC) including India. Case records of children with newly diagnosed extraocular RB from January 2013 to August 2016 treated at our unit were analysed for clinical profile, treatment, and outcome. Over the 44 month study period, 91 children were diagnosed with RB, out of which 41 had extraocular disease. While 26 children had extraocular spread limited to orbit (IRSS stage III), 15 had a distant spread to brain (IRSS stage IV). Median lag period for diagnosis was eight months. Treatment abandonment rates were 38.5% and 46.6% in International Retinoblastoma Staging System (IRSS) stage III and IV respectively. With a median follow up of 31.5 months, the projected overall survival for IRSS III at one, two, and three years was 87.5%,...