Evolving trends in retinoblastoma management: A 16-year clinicopathological analysis of enucleated eyes

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Abstract: PURPOSE: New treatments for advanced retinoblastoma (RB) have offered alternatives to primary enucleation. We assessed the impact of these therapies on the indications for enucleation and the histopathological findings in enucleated eyes with RB. MATERIALS AND METHODS: Eyes of all patients who underwent enucleation for RB at a single institution between January 2005 and August 2021 were included. Data collected retrospectively included demographics, clinical and pathologic staging, pathologic findings, and management. Statistical analysis included Kendall’s τ b , Pearson χ 2 , and Cramér’s V. RESULTS: There were 254 eyes from 252 patients with information available for review. Annual enucleations decreased between 2005 and 2008 at a rate of 4.2 enucleations/year, increased from 2008 to 2013 at a rate of 2.7 enucleations/year and decreased from 2013 to 2019 at a rate of 1.5 enucleations/year, reflecting changes in RB therapies. When compared to earlier years, the eyes enucleated in recent years were more likely to be enucleated for patient symptoms ( P < 0.001) and insufficient view ( P = 0.019), were more likely to have prior treatment ( P < 0.001), had lower tumor stage ( P = 0.010) and grade ( P = 0.006), contained no viable tumor ( P < 0.001), and were phthisical ( P = 0.003). Five of 252 patients (2%) developed metastases; one of these patients had no viable tumor in a previously treated enucleated eye. CONCLUSION: Therapeutic innovations shifted the management of RB from primary enucleation in favor of eye salvage. Enucleated eyes show less viable tumor and disease severity but more intraocular degeneration, emphasizing the importance of skilled pathologic interpretation.

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