Outcome of retinoblastoma patients treated according to the University Hospital of Siena guidelines

Purpose To analyze the results of Retinoblastoma (Rb) treatment according to the University Hospital of Siena guidelines in terms of complete ophthalmoscopic remission (COR) of disease. Methods A total of 109 eyes of 87 patients were enrolled from September 2013 to November 2016. The mean patient age at diagnosis was 22.8 months (± sd 20.5 months). Sixtysix/109 eyes (60.55% of cases) were unilateral, 42 (38.53%) bilateral. Thirtyfive/109 (32.11%) were classified as low stage (A-C of ICRB classification), 36 (33.03%) as D stage and 31 (28.44%) as E stage. Interventions: Conservative treatment with intravenous (IV) chemotherapy (CHT) and/or intrarterial (IA) CHT, both as first or second line therapies; primary or secondary enucleation. Results Fourtysix/109 eyes (42.20%) received primary IV CHT, 40 (36.70%) primary IA CHT and 22 (20.1%) were enucleated at diagnosis. Twentyfive eyes (22.9%) were treated with secondary IA CHT (for failure of IV CHT, the majority being bilateral cases), 4 (3.6%) with second line IV CHT (mainly thermo-CHT after IA CHT). Twentythree/109 eyes (21.1%) were secondary enucleated. Globe salvage with COR of disease has been obtained by 53/109 eyes (49% of all cases, 60.9% of the treated ones), in particular: by the 32.5% of the eyes treated with primary IV CHT, by the 52.5% of those treated with primary IA CHT and by the 60% of the eyes treated with secondary IA CHT. Mean follow-up is of 21.9 months (± sd 16.9 months). Conclusions These results confirm the efficacy of our guidelines for Rb treatment. Particularly we obtained a better ocular salvage rate (p > χ2 = 0.0007, Mantel Cox Comparisons) for the cases treated with secondary IA CHT than with primary IA CHT. This observation demonstrates the efficacy of IA CHT even in recurrences of Rb after primary IV CHT, if early detected with a close follow-up.