Recent developments in the management of retinoblastoma.

Abstract

The management of retinoblastoma has gradually changed over the past few decades. There is a trend away from enucleation and external beam radiotherapy toward focal conservative treatments. This is primarily because of earlier detection of the disease and more focused treatment modalities. Enucleation is still employed for retinoblastoma that fills most of the eye, especially when there is a concern for tumor invasion into the optic nerve or choroid. After enucleation, an integrated orbital implant, provides improved motility and appearance of the prosthesis. External beam radiotherapy continues to be an important method of treating less advanced retinoblastoma, especially when there is diffuse vitreous or subretinal seeding. Plaque radiotherapy is useful for controlling small- to medium-sized retinoblastomas, even those with focal vitreous seeds. Tumors that recur after failure of other methods are often suitable for plaque treatment. When plaque radiotherapy is employed in a child receiving chemotherapy, eventual radiation retinopathy can occur. Cryotherapy and photocoagulation provide excellent control of selected small tumors. Advanced laser delivery systems, particularly those that have been adapted to the indirect ophthalmoscope, have facilitated the visualization for treatment of tumors. Thermotherapy is the newest focal method for retinoblastoma. When combined with chemotherapy, thermotherapy provides satisfactory tumor control, leaving the child with a reasonably small scar, thus preserving more vision. Chemoreduction, using intravenous or subconjunctival routes, is often employed to reduce initial tumor volume and thus allow for focal treatment to eradicate the residual smaller tumor. Many children with advanced retinoblastoma can be spared external beam radiotherapy and enucleation mostly as a result of chemoreduction and focal methods. Chemoreduction combined with cryotherapy, thermotherapy, and plaque radiotherapy plays an important role in the current management of many children with retinoblastoma.