The management of advanced intraocular retinoblastoma is evolving rapidly, with enucleation progressively being supplanted by globe-sparing treatments, such as intra-arterial chemotherapy.1 While the battle to save more eyes is admirable, the judicious use of these new treatments is essential to avoid compromising the high survival rates that have been achieved in the United States and other developed countries.2 Retinoblastoma cell invasion of the optic nerve and choroid—2 of the most important risk factors for metastasis—are considered by many experts to be indications for adjuvant systemic chemotherapy.2 Yet, these factors cannot be assessed accurately without histopathologic examination of the enucleated eye. Approximately 10% to 15% of advanced intraocular retinoblastoma cases harbor high-risk histopathologic features,3 which go undetected with globe-sparing treatments that may not be as effective as enucleation in preventing metastasis in high-risk cases. Currently, there is no consensus on the best treatment of advanced intraocular retinoblastoma, with the rate of primary enucleation ranging from 0% to 100% among referral centers around the world.4
Harbour JW. Liquid Biopsy in Retinoblastoma. JAMA Ophthalmol. 2017;135(11):1231. doi:https://doi.org/10.1001/jamaophthalmol.2017.4094
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: