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C. E. Fernandes, A. C. Schefler, J. Reichbach, A. Podda, J. A. Davis, O. A. Alvarez, J. C. Barredo, N. Lewis, E. White, T. Murray; Chemotherapy Plus Cyclosporine A for the Treatment of Intraocular Retinoblastoma. Invest. Ophthalmol. Vis. Sci. 2009;50(13):1692.
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A retrospective analysis of 41 patients diagnosed with bilateral Rb from Dec 1996 to Jan 2006 was performed. Only eyes with intraocular disease (76 eyes) were included in this analysis. Patients were treated with local therapy and chemotherapy consisting of vincristine, carboplatin and etoposide with or without cyclosporine A (CEV or CEVC) every 3-4 weeks. Before each cycle of chemotherapy ophthalmologic examination under anesthesia was performed and active tumor and seeding were treated with local ablation using large spot size custom diode laser therapy. Eyes in which enucleation was planned at diagnosis were excluded from this analysis.
Most patients received 9 cycles of chemotherapy. Sixty percent (46/76) of the eyes were treated with chemotherapy and CSA. The eye salvage rate for eyes classified by the International Classification of Retinoblastoma (ICRB) as groups A, B, C and D was 100%. The eye salvage rate for the 21 eyes classified as ICRB group E was 29%. No difference in salvage rate was observed for group E eyes treated with or without CSA.
Salvage rates for ICRB groups A-D are outstanding with current treatments. The addition of CSA in patients with stage E eyes did not provide further benefits. Aggressive focal control and chemotherapy beginning at diagnosis may account for the excellent EFS in group A, B, C and D eyes.
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