Abstract
PurposeTo report on the influence of ophthalmic artery chemosurgery (OAC) on enucleation rates, ocular and patient survival from metastasis and impact on practice patterns at Memorial Sloan Kettering for children with advanced intraocular unilateral retinoblastoma.Patients and MethodsSingle-center retrospective review of all unilateral retinoblastoma patients with advanced intraocular retinoblastoma treated at MSKCC between our introduction of OAC (May 2006) and December 2014. End points were ocular survival, patient survival from metastases and enucleation rates.Results156 eyes of 156 retinoblastoma patients were included. Primary enucleation rates have progressively decreased from a rate of >95% before OAC to 66.7% in the first year of OAC use to the present rate of 7.4%. The percent of patients receiving OAC has progressively increased from 33.3% in 2006 to 92.6% in 2014. Overall, ocular survival was significantly better in eyes treated with OAC in the years 2010–2014 compared to 2006–2009 (p = 0.023, 92.7% vs 68.0% ocular survival at 48 months). There have been no metastatic deaths in the OAC group but two patients treated with primary enucleation have died of metastatic disease.ConclusionOAC was introduced in 2006 and its impact on patient management is profound. Enucleation rates have decreased from over 95% to less than 10%. Our ocular survival rate has also significantly and progressively improved since May 2006. Despite treating more advanced eyes rather then enucleating them patient survival has not been compromised (there have been no metastatic deaths in the OAC group). In our institution, enucleation is no longer the most common treatment for advanced unilateral retinoblastoma.
Highlights
Enucleation has always been the most common treatment for both unilateral and bilateral retinoblastoma[1]
Primary enucleation rates have progressively decreased from a rate of >95% before ophthalmic artery chemosurgery (OAC) to 66.7% in the first year of OAC use to the present rate of 7.4%
Ocular survival was significantly better in eyes treated with OAC in the years 2010–2014 compared to 2006–2009 (p = 0.023, 92.7% vs 68.0% ocular survival at 48 months)
Summary
Enucleation has always been the most common treatment for both unilateral and bilateral retinoblastoma[1]. It has resulted in very good patient outcomes, it is a surgical procedure that leaves a permanent cosmetic reminder and deprives the patient of any possible sight in the eye(s). External beam irradiation was the first treatment technique that allowed clinicians to save an eye–often with useful vision. Introduced more than 100 years ago it was the only treatment that allowed salvaging of eyes with advanced disease. Chemotherapy alone was rarely curative, when combined with focal techniques such as laser, cryotherapy or brachytherapy (and occasionally external beam irradiation) many eyes could be salvaged[9]. Eyes with advanced retinoblastoma were salvaged in fewer than 50% of cases[8] and side effects, including secondary Acute Myeloid Leukemia were worrisome[10]
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