Retinoblastoma management: achieving high standards of care

The current management of retinoblastoma involves chemotherapy by the intravenous, intra-arterial, intravitreous or intra-aqueous delivery routes. These routes can adequately control solid tumour, subretinal seeds, vitreous seeds or aqueous seeds. Intravenous chemotherapy using vincristine, etoposide and carboplatin is commonly used for retinoblastoma control. According to the International Classification of Retinoblastoma, intravenous chemotherapy provides globe salvage with tumour control in 96% of Group A, 90% of Group B and Group C, 68% of Group D and 32% of Group E eyes. Intra-arterial chemotherapy, using melphalan, topotecan and/or carboplatin with delivery from the femoral artery up to the ophthalmic artery to the eye, is particularly used for unilateral retinoblastoma. This alternative has more localised ocular complications but offers more powerful tumour control. Intravitreal chemotherapy, using melphalan and topotecan, can control vitreous seeding, but most specialists employ high-dose topotecan for control of vitreous seeds and some solid tumours and even subretinal seeds. Intra-aqueous chemotherapy, using melphalan or topotecan and can be effective for aqueous seeding. There is still a role for plaque radiotherapy in non-chemotherapy-responsive tumours and enucleation for massive disease that doesn't fully respond to chemotherapy or demonstrates high-risk disease. The management of retinoblastoma has evolved to the current day with several routes of chemotherapy delivery.