pediatrics
Retinoblastoma
Treatment of Retinoblastoma
For advanced unilateral cancer, enucleation For less advanced unilateral cancer, sometimes chemotherapy and/or local-control treatments For bilateral cancer, photocoagulation, intra-arterial chemotherapy, or unilateral enucleation with photocoagulation, cryotherapy and irradiation of the other eye Systemic chemotherapy The goal of retinoblastoma treatment should be cure, but attempts to preserve as much vision as possible are appropriate. The treatment approach depends on the size of the tumor (more or less than 3 mm in dimension/thickness), spread to surrounding areas, and functionality of the eye. A multidisciplinary team is highly recommended and should include a pediatric ophthalmologist with expertise in retinoblastoma, a pediatric oncologist, and a radiation oncologist. Advanced unilateral retinoblastoma (large tumor with evidence of disease extension) is managed by enucleation with removal of as much of the optic nerve as possible. In less advanced cases where preservation of vision is a possibility, conservative eye salvage approaches with chemotherapy and/or local-control treatments can be considered. For patients with bilateral cancer, vision can usually be preserved. Options include bilateral photocoagulation, intra-arterial chemotherapy, or unilateral enucleation and photocoagulation, cryotherapy, and irradiation of the other eye. Radiation therapy is by external beam or, for very small cancers, brachytherapy (attachment of a radioactive plaque to the eye wall near the cancer). Systemic chemotherapy, such ascarboplatin,etoposide, andvincristine, orcyclophosphamideplusvincristine, may be helpful to reduce the size of large cancers to allow for the use of other additional therapies (eg, cryotherapy, laser hyperthermia), to treat bilateral tumors, or to treat cancer that has disseminated beyond the eye. However, chemotherapy alone can seldom cure this cancer. Ophthalmologic re-examination of both eyes and retreatment, if necessary, are required at 2- to 4-month intervals. 