Bilateral retinoblastoma: clinical presentation, management and treatment

Management of retinoblastoma (Rb), the most common intraocular malignant tumor in childhood, is tailored to each individual case and based on the overall situation. We present a case of bilateral Rbs in a 4-month-old girl, referred to our center for bilateral leukocoria. In the right eye, the optic disc was partially visible, and three large foci of retinoblastoma were noted adjective in the vitreous cavity with satellite retinal detachment. The macula was obscured by the tumors. The tumor was therefore classified as group D (International classification of retinoblastoma), and thus underwent intravenous chemotherapy with the standard three-agent protocol of ifosfamide, carboplatin and etoposide (ICE protocol) delivered monthly for six cycles, regressing with a type I calcified pattern. The left eye presented three multifocal yellow-white retinal masses, with a total retinal detachment, and secondary glaucoma. The lesions were classified as group E and, therefore, taking the results obtained by Shields et al. in group E tumors into consideration, underwent enucleation. This case clearly shows that this cancer is curable if detected at a stage in which it is still contained within the retina, subretinal space or vitreous, and that the management of Rb relies on an experienced team of ocular oncologists, pediatricians and pediatric ophthalmologists working together for the single goal of saving the child's life.