Increased clinical risk factors and absence of leucocoria in late-onset retinoblastoma: a comparative cohort study.
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AIMS: To characterise the clinicopathological features, treatment outcomes and prognostic factors in late-onset retinoblastoma (LoRB) (≥5 years) compared to classical-onset cases (<5 years). METHODS: This retrospective cohort study included 21 cases of LoRB and 84 matched classical-onset cases diagnosed between April 2016 and December 2023 at two tertiary retinoblastoma (RB) centres. Data on clinical presentations, histopathological features, treatment modalities and outcomes were collected and analysed between two groups. A subgroup analysis was performed based on presenting symptoms in the late-onset group. RESULTS: During the study period, 21 patients with LoRB were diagnosed, accounting for 5.43% of all RB cases. The median age at diagnosis was 80 months. All late-onset cases presented unilaterally and were in International Intraocular Retinoblastoma Classification groups D and E. Compared with the control group, late-onset patients demonstrated significantly longer diagnostic lag times (p=0.008), a markedly lower rate of leucocoria as the presenting symptom (p<0.001), a higher rate of vitreous seeding (p=0.03) and subretinal fluid (p=0.04), and shorter median globe salvage times (p=0.04). Late-onset patients with atypical onset symptoms experienced longer diagnostic lag times (p=0.05), more group E eyes at diagnosis (p=0.03) and shorter median globe salvage times (p=0.05) compared with those with classical symptoms (leucocoria and strabismus). CONCLUSION: LoRB presents distinct clinical features and more severe manifestations, highlighting the necessity for early recognition and timely intervention of this subtype. Our findings contribute to a more comprehensive understanding of the relationship between the age of onset and RB behaviour.