Clinical features and treatment outcomes in children with retinoblastoma at a single tertiary referral center in Turkey.
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PURPOSE: Retinoblastoma is the most common primary intraocular malignancy of childhood. The goal of this study is to evaluate the clinical characteristics, treatment approach, patient survival and ocular survival rates in children with retinoblastoma in Turkey. METHODS: The medical records of 297 patients (403 eyes) diagnosed with retinoblastoma between 1983 and 2017 were retrospectively evaluated. RESULTS: Twenty-eight eyes were in group A (6.9%), 65 eyes in group B (16.1%), 27 eyes in group C (6.7%), 82 eyes in group D (20.3%), and 165 eyes in group E (40.9%). The globe preservation rate was 30.9% in cases with unilateral involvement, and the preservation rate for at least one eye in cases with bilateral involvement was 67%. The five-year eye preservation rates were as follows: 100% in group A, 96% in group B, 90.9% in group C, 68.4% in group D, and 7.8% in group E. Furthermore, tumor stage and laterality significantly impacted globe survival rates (P<0.001 for both). The five-year survival rate for patients was 96.6% according to Kaplan-Meier analysis. Among the 10 patients who died, five families discontinued treatment and follow-up after recurrences; three patients succumbed to a secondary tumor. Of the two patients receiving systemic chemotherapy, one experienced a traumatic subarachnoid hemorrhage, and the other developed thrombocytopenia, which led to cerebral hemorrhage. CONCLUSION: The results from our center demonstrate significant advancements in RB treatment, reflecting progress toward the same standards as in developed countries. Notably, enucleation rates have decreased over the years, particularly in unilateral cases. This improvement is likely linked to enhancements in the healthcare system, better education of medical professionals, and greater community awareness. The use of modern treatments and a multidisciplinary approach in developing countries contributes to high patient and ocular survival rates.