Abstract

PurposeTo report associations between disease- and treatment-related variables and rates of recurrence-free survival and ocular survival in eyes treated with ophthalmic artery chemosurgery (OAC) for retinoblastoma.DesignPre-post study.SubjectsAll eyes treated with OAC for retinoblastoma at Memorial Sloan Kettering Cancer Center between May 2006 and February 2017.MethodsThis retrospective review included 452 retinoblastoma eyes treated with OAC. The Kaplan-Meier method was used to estimate recurrence-free survival (RFS), progression-free survival (PFS) and ocular survival (OcS), and Cox regression was used to estimate hazard ratios. Eyes treated in the pre-intravitreous chemotherapy era were analyzed separately from eyes treated in the intravitreal era.Main outcome measuresRecurrence-free survival, ocular survival, associations with risk of recurrenceResultsDisease and treatment characteristics were recorded over a median 23.6 month follow-up. One-year OcS, PFS and RFS were 96% (95% CI 93–99%), 88% (95% CI 88–94%) and 74% (95% CI 67–81%) in the pre-intravitreal era and 96% (95% CI 94–99%), 93% (95% CI 89–96%) and 78% (95% CI 72–83%) in the intravitreal era, respectively. Presence of vitreous seeds was associated with increased risk of recurrence in the pre-intravitreal era but not in the intravitreal era. Longer time interval between OAC sessions was associated with increased risk of recurrence and majority OAC access via the ophthalmic artery was associated with decreased risk of recurrence in both eras.ConclusionsApproximately a quarter of eyes initially treated with ophthalmic artery chemosurgery develop recurrent disease, with the majority of recurrences within the first year following completion of OAC. Despite this, these eyes have a very good chance of salvage. In eyes with vitreous seeds at presentation, intravitreal injections are useful in minimizing future vitreous recurrence. Eyes that receive the majority of drug infusions via non-ophthalmic artery routes or greater interval between OAC are more likely to recur and might warrant closer monitoring.

Highlights

  • Retinoblastoma is the pediatric solid cancer with the highest survival rate in developed countries (>99% in some series), this was historically accomplished by removing one or both eyes. [1] For most of the 20th century, the only way to salvage eyes with advanced intraocular disease was external beam irradiation. [2] Radiation did salvage 25% of such eyes and often with useful vision, but at least a quarter required additional, subsequent focal treatments. [3] It is impossible to know from the published literature if this was because of persistent disease or local recurrences, because such information was not tabulated

  • One-year ocular survival (OcS), progression-free survival (PFS) and recurrence-free survival (RFS) were 96%, 88% and 74% in the pre-intravitreal era and 96%, 93%

  • A quarter of eyes initially treated with ophthalmic artery chemosurgery develop recurrent disease, with the majority of recurrences within the first year following completion of OAC

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Summary

Introduction

Retinoblastoma is the pediatric solid cancer with the highest survival rate in developed countries (>99% in some series), this was historically accomplished by removing one or both eyes. [1] For most of the 20th century, the only way to salvage eyes with advanced intraocular disease was external beam irradiation. [2] Radiation did salvage 25% of such eyes and often with useful vision, but at least a quarter required additional, subsequent focal treatments. [3] It is impossible to know from the published literature if this was because of persistent disease or local recurrences, because such information was not tabulated. [1] For most of the 20th century, the only way to salvage eyes with advanced intraocular disease was external beam irradiation. [2] Radiation did salvage 25% of such eyes and often with useful vision, but at least a quarter required additional, subsequent focal treatments. [5] Ninety-nine percent of eyes treated with systemic chemotherapy required additional laser, cryotherapy, brachytherapy or external beam irradiation (and even enucleation), but it is impossible to know how many of these were “routine” additional treatments and how many were treated because of local recurrences. The introduction of intra-arterial chemotherapy (ophthalmic artery chemosurgery, OAC) in 2006 allowed the majority of advanced eyes to be salvaged. [7] With time and experience, a majority of eyes treated with OAC were salvaged without compromising patient survival or vision. The introduction of intra-arterial chemotherapy (ophthalmic artery chemosurgery, OAC) in 2006 allowed the majority of advanced eyes to be salvaged. [6] Within five years of its introduction, many centers worldwide were using it as first-line treatment for advanced eyes. [7] With time and experience, a majority of eyes treated with OAC were salvaged without compromising patient survival or vision. [8] Some blind eyes even regained visual potential after OAC. [9]

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