A Systematic Review of Long-Term Neurocognitive Outcomes in Paediatric Retinoblastoma Survivors

Background Retinoblastoma (RB) is the most common intraocular malignancy in children. Survivors may experience long-term ophthalmic and systemic morbidity. This review aims to evaluate the neurocognitive outcomes of paediatric RB survivors. Methods Guided by the PRISMA guidelines, a systematic search was conducted across five databases: PubMed, Scopus, Embase, Web of Science and Cochrane Library. Inclusion criteria were: (1) studies published between January 2015 and June 2025, (2) full-text availability, (3) English language, (4) human subjects aged ≤18 years at diagnosis, and (5) reporting on neurocognitive outcomes ≥2 years RB post-treatment. From an initial pool of 971 papers, 5 were selected for review. Results RB survivors exhibit neurocognitive impairments across multiple domains, including intelligence, verbal memory, adaptive behaviour, academic performance, and facial processing. Intelligence and verbal memory impairments were notable, particularly in survivors with 13q deletion syndrome, who showed persistent deficits. Adaptive behaviour declined early but improved by age 10 years, with racial differences suggesting socioeconomic influences. Academic challenges and developmental delays were prevalent, requiring educational support. Early monocular enucleation impaired facial processing due to absent stereopsis, disrupting visual-cognitive development. These neurocognitive deficits are often reported in association with RB therapy (surgery, chemotherapy and radiation) and impact both educational and functional outcomes. Conclusion These findings highlight the importance of monitoring and targeted early interventions to support children treated for RB and improve long-term outcomes, though the limited evidence base calls for further research.