Abstract
ObjectiveReport on the 7-year experience with bilateral ophthalmic artery chemosurgery (OAC-Tandem therapy) for bilateral retinoblastoma.DesignRetrospective, single institution study.Subjects120 eyes of 60 children with bilateral retinoblastoma treated since March 2008.MethodsRetrospective review of all children treated at Memorial Sloan Kettering with bilateral ophthalmic artery chemosurgery (Melphalan, Carboplatin, Topotecan, Methotrexate) delivered in the same initial session to both naïve and previously treated eyes.Main Outcome MeasuresOcular survival, metastatic disease, patient survival from metastases, second cancers, systemic adverse effects, need for transfusion of blood products, electroretinogram before and after treatment.Results116 eyes were salvaged (4 eyes were enucleated: 3 because of progressive disease, 1 family choice). Kaplan Meier ocular survival was 99.2% at one year, 96.9% at 2 and 3 years and 94.9% for years 4 through 7. There were no cases of metastatic disease or metastatic deaths with a mean follow-up of 3.01 years. Two children developed second cancers (both pineoblastoma) and one of them died. Transfusion of blood products was required in 3 cases (4 transfusions), 1.9%. Two children developed fever/neutropenia requiring hospitalization (0.95%). ERGs were improved in 21.6% and unchanged after treatment in 52.5% of cases (increase or decrease of less than 25μV).ConclusionsBilateral ophthalmic artery chemosurgery is a safe and effective technique for managing bilateral retinoblastoma-even when eyes are advanced bilaterally, and if both eyes have progressed after systemic chemotherapy. Ocular survival was excellent (94.9% at 8 years), there were no cases of of metastatic disease and no deaths from metastatic disease, but children remain at risk for second cancers. In 21.6% of cases ERG function improved. Despite using chemotherapy in both eyes in the same session, systemic toxicity was low.
Highlights
Ophthalmic artery chemosurgery (OAC) was introduced almost 10 years ago for the treatment of retinoblastoma[1] and has been performed in more than 45 countries worldwide for unilateral retinoblastoma.[2]
There were no cases of metastatic disease or metastatic deaths with a mean follow-up of 3.01 years
ERGs were improved in 21.6% and unchanged after treatment in 52.5% of cases
Summary
Ophthalmic artery chemosurgery (OAC) was introduced almost 10 years ago for the treatment of retinoblastoma[1] and has been performed in more than 45 countries worldwide for unilateral retinoblastoma.[2] In a recent survey it was the first-line choice for the majority of retinoblastoma centers worldwide for patients with advanced unilateral disease.[3] The technique has been successful for naïve eyes[4,5] and eyes that failed all prior therapies.[6,7] It has enabled the majority of advanced eyes that were previously managed with enucleation to be saved–[4,8,9,10] sometimes with useful vision–[10,11] without compromising patient survival. Fewer than 10 such cases have been reported in the literature since our description of the technique
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