Purchase this article with an account.
T.G. Murray, S. Toledano, A. Markoe, A.C. Schefler; Failure Analysis for Globe Conservation in Advanced Intraocular Retinoblastoma . Invest. Ophthalmol. Vis. Sci. 2005;46(13):1111.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
Purpose: The purpose of this study was to utilize failure analysis to determine the risk factors associated with globe conservation failure for eyes with advanced intraocular retinoblastoma (RE Stage V) undergoing globe salvage primary therapy including systemic chemoreduction or external beam radiotherapy, coupled with transpupillary focal laser tumor ablation. Methods: This study was an IRB approved, retrospective analysis of 167 eyes of 103 consecutive patients presenting with advanced intaocular retinoblastoma and prospectively treated within the pediatric ocular oncology division of the Bascom Palmer Eye Institute with planned primary globe conserving therapy between 1991 and 2002. Recorded variables included age at presentation, race, sex, and family history.All eyes were RE stage V. Treatment variables were chemotherapy, laser thermotherapy, or external beam radiotherapy. Failure was defined as requirement for enucleation, development of metastatic disease or death. All eyes were clinically staged at time of enucleation, histopathologically reviewed and analyzed within the ocular oncology research laboratory. Results:Failure analysis identified eyes undergoing enucleation as no patient developed metastatic disease or died during the study period (mean follow–up: 78 months). This study identified seventeen eyes (17/167, 11%) enucleated after planned globe conservation. No statistical difference was noted between eyes treated with external beam radiotherapy or systemic chemoreduction with focal tumor ablation (p > .35).All eyes failed with progressive vitreous seeding with/or without subretinal seeding. No eye failed from progressive retinal disease, extraocular extension or new tumor development. Conclusions: Globe conservation with advanced retinoblastoma yields improving ocular salvage rate when external beam radiotherapy and/or systemic chemotherapy are coupled with aggressive local tumor treatment. Failure analysis documents primary indications for enucleation to be advanced intraocular retinoblastoma (RE Stage Vb)with the failure mode being progressive vitreous and/or subretinal tumor seeding. Further focus on treatment of vitreous and/or subretinal seeding is likely to enhance globe retention in advanced retinoblastoma.
This PDF is available to Subscribers Only