Chemoreduction plu focal therapy for retinoblatoma: factor predictive of need for treatment with external beam radiotherapy or enucleation

PURPOE: To report the reult of chemoreduction and focal therapy for retinoblatoma with determination of factor predictive of the need for treatment with external beam radiotherapy or enucleation DEIGN: Interventional cae erie METHOD: One-hundred three patient with retinoblatoma (158 eye with 364 tumor) at the Ocular Oncology ervice at Will Eye Hopital of Thoma Jefferon Univerity in conjunction with the Diviion of Oncology at Children’ Hopital of Philadelphia from June 1994 to Augut 1999 were enrolled for thi propective clinical trial. The patient received treatment for retinoblatoma with ix planned cycle (one cycle per month) of chemoreduction uing vincritine, etopoide, and carboplatin combined with focal treatment (cryotherapy, thermotherapy, or plaque radiotherapy). The two main outcome meaure after chemoreduction and focal therapy were the need for external beam radiotherapy and the need for enucleation. The clinical feature at the time of patient preentation were analyzed for impact on the main outcome meaure uing a erie of Cox proportional hazard regreion. REULT: Uing Reee-Ellworth (RE) taging for retinoblatoma, there were nine (6%) eye with group I dieae, 26 (16%) eye with group II dieae, 16 (10%) eye with group III dieae, 32 (20%) eye with group IV dieae, and 75 (48%) eye with group V retinoblatoma. All eye howed initial favorable repone with tumor regreion. The median follow-up wa 28 month (range, 2–63 month). Failure of chemoreduction and need for treatment with external beam radiotherapy occurred in 25% of eye at 1 year, 27% at 3 year, and no further increae at 5 year. More pecifically, external beam radiotherapy wa neceary at 5 year in 10% of RE group I–IV eye and 47% of RE group V eye. Multivariate factor predictive of treatment with external beam radiotherapy included non-Caucaian race, male ex, and RE group V dieae. Failure of chemoreduction and the need for treatment with enucleation occurred in 13% eye at 1 year, 29% at 3 year, and 34% at 5 year. More pecifically, enucleation wa neceary in 15% of RE group I–IV eye at 5 year and in 53% of RE group V at 5 year. Multivariate factor predictive of treatment with enucleation included patient age older than 12 month, ingle tumor in eye, and tumor proximity to foveola within 2 mm. Overall, of the 158 eye, 50% required external beam radiotherapy or enucleation and 50% were uccefully managed without thee treatment. No patient developed retinoblatoma metatai, pinealoblatoma, or econd malignant neoplam over the 5-year follow up. CONCLUION: Chemoreduction offer atifactory retinoblatoma control for RE group I–IV eye, with treatment failure neceitating additional external beam radiotherapy in only 10% of eye and enucleation in 15% of eye at 5-year follow-up. Patient with RE group V eye require external beam radiotherapy in 47% and enucleation in 53% at 5 year.