Retinoblastoma survival trend: a 30-year analysis from a referral single center in Iran

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BACKGROUND: The treatment of retinoblastoma (Rb) has undergone significant improvements over the last century. This study aims to assess the trend of enucleation and mortality in retinoblastoma patients during the last 3 decades. METHODS: The study utilized data from the referral center for ocular oncology in Rasool Akram Hospital, Tehran, Iran. It included all patients diagnosed with Rb from August 1991 to December 2018. The study investigated the trend of enucleation and mortality during three-time intervals: before 2001 (T1), during 2001-2007 (T2), and 2008-2018 (T3). Additionally, it assessed the trend of enucleation and mortality based on laterality, age and presentation of Rb (strabismus and leukocoria). RESULTS: The incidence of enucleation decreased significantly from T1 to T3 (74-41%) during the study period (p-value < 0.001). Pairwise comparisons between T1 and T3 revealed a significant decrease in the incidence of enucleation (74% vs. 41%, p-value < 0.001). The study also demonstrated a significant reduction in the incidence of enucleation when comparing T2 to T3 (60% vs. 41%, p-value < 0.001). Comparing time intervals, there was no significant difference between T2 and T3 regarding the incidence of death (4% vs. 1%), but both intervals had statistically significant lower death rates compared with T1 (26%, both p-values < 0.001). CONCLUSION: This study revealed that Introduction of systemic chemotherapy as mainstay of Rb treatment, has led to a significant reduction in mortality and morbidity rates. The incorporation of targeted chemotherapy has further decreased the need for enucleation, but it has not substantially impacted the mortality rate. Unfortunately, in spite of reduction trend in enucleation, systemic and targeted chemotherapy was unable to save the affected globe in nearly half of the patients, even when the malignancy was diagnosed in the earlier stages.

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