Practical Approach to Management of Retinoblastoma

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Abstract
Objective To present a simplified approach to management of retinoblastoma usingbasic clinical features. Design In a prospective, nonrandomized, single-center clinical trial, 158 eyesof 103 patients with retinoblastoma were managed with 6 cycles of chemoreduction(vincristine sulfate, etoposide, and carboplatin). The eyes were classifiedaccording to the Reese-Ellsworth classification and were also grouped on thebasis of clinical features as follows: group 1, tumor only; group 2, tumorplus subretinal fluid; group 3, tumor plus focal seeds (3a, focal subretinalseeds; 3b, focal vitreous seeds); group 4, tumor plus diffuse seeds (4a, diffusesubretinal seeds; 4b, diffuse vitreous seeds); and group 5, neovascular glaucomaor invasive retinoblastoma. Main Outcome Measure Treatment success (avoidance of enucleation and external beam radiotherapy). Results According to the Reese-Ellsworth classification, chemoreduction wassuccessful in 100% of group Ia, 100% of group Ib, 86% of group IIa, 100% ofgroup IIb, 91% of group IIIa, 100% of group IIIb, 50% of group IVa, 77% ofgroup IVb, 50% of group Va, and 27% of group Vb. There was erratic correlationof the Reese-Ellsworth classification with treatment success. In contrast,the simplified grouping system displayed a smooth, nonerratic correlationfor treatment success, with 100% success for group 1, 91% for group 2, 59%for group 3, and 12% for group 4 (group 5 always managed by primary enucleation).When all 6 subcategory groups were analyzed, there was consistent correlationfor treatment success of 100% for group 1, 91% for group 2, 68% for group3a, 54% for group 3b, 17% for group 4a, and 11% for group 4b. Conclusion This practical approach to retinoblastoma using basic clinical featuresis predictive of treatment success for eyes in which modern conservative therapyfor retinoblastoma is used.