Combined Chemoreduction and Adjuvant Therapy for Management of Intraocular Retinoblastoma

Purpose: The aim of the current study is to investigate the effectiveness of combined chemoreduction and local ophthalmic therapy for treatment of intraocular retinoblastoma with the goal of avoiding enucleation and/or external beam radiotherapy (EBRT).Patients and Methods: This is a prospective non - randomized study. Fifty-seven eyes were followed in 43 children with intraocular retinoblastoma who attended to the pediatric unit of Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) during the period from March 2002till March 2004. Eiligible patients were treated with a sixcycle protocol of vincristine, etoposide, and carboplatin (VEC). When maximum retinal tumors regression was achieved, adjuvant treatment (thermotherapy or cryotherapy) was delivered without randomization to the smallest possible tumor volume in an effort, if possible, to avoid enucleation and/or EBRT.Results: The mean age of the patients at presentation was 27.69 months (range 1-96 months). The Reese-Ellsworth classification included, group I non, group II in 6 eyes (10.5%), group III in 13 eyes (22.8%), group IV in 21 eyes (36.9%), and group IV in 17 eyes (29.8%). In 35 eyes combined chemoreduction and local ophthalmic therapy were successful in terms of avoidance of enucleation and/or EBRT, with a success (globe salvage) rate of 61.4%. All Reese-Ellsworth group II and III eyes responded to the conservative approach, whereas only 12/21 (57.1%) and 4/17(23.5%) of group IVand V eyes respectively, responded. Seven eyes (12.3%) required EBRT, 5 eyes (8.8%) required enucleation and 10 eyes (17.5%) required a combination of EBRT and enucleation. Event-free survival (i.e, free of EBRT or enucleation) for the study population at one year was 50.8%. Conclusion: In retinoblastoma patients with Reese-Ellsworth eye groups 1, 2, or 3, systemic chemotherapy used with local ophthalmic therapies can eliminate the need for enucleation or EBRT without significant systemic toxicity. More effective therapy is required for Reese-Ellsworth eye groups 4 and 5.