Eye Cancers (Retinoblastomas) Support Group

Retinoblastoma is a cancer of the retina. Development of this tumor is initiated by mutations that inactivate both copies of the RB1 gene, which codes for the retinoblastoma protein. Until recently the only treatment was to remove the affected eyeball before the cancer spread. Chemotherapy is the treatment of choice for most unilateral cases. However with locally advanced disease external beam radiation may be needed and if both eyes are involved enucleation may be the only option. Affected children in developing countries present with advanced features and usually die of metastatic spread. In its initial stages, retinoblastoma is very similar to Coats disease, a non-cancerous retina disease. Coats' disease should be ruled out before enucleation is done. A mis-diagnosis of retinoblastoma accounts for the greatest number of Coats' disease eyes being enucleated. Many children with bilateral retinoblastoma can be treated with a preservation attempt. Tumor chemoreduction with carboplatin and other drugs may reduce the tumor volume making them amenable to local therapies