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Retinoblastoma is a cancerous tumor of the RETINA that most often occurs in children. Most retinoblastomas are hereditary and develop in early childhood, usually by age four. Some retinoblastomas are the result of new germline mutations though are not hereditary. About 70 percent of retinoblastomas involve only one EYE. Treatment in such cases is surgery to remove the EYE (ENUCLEATION), with placement of a PROSTHETIC EYE for cosmetic reasons.
When retinoblastoma is bilateral (involves both eyes), treatment attempts to save vision while eradicating the CANCER. Treatment for bilateral retinoblastoma may include enucleation of one eye and cryotherapy or photocoagulation to reduce as much as possible the tumor in the other eye, with follow-up CHEMOTHERAPY or RADIATION THERAPY. Treatment is successful in about 90 percent of children when doctors detect the tumor before it metastasized beyond the eye. However, about 70 percent will experience second retinoblastomas by adulthood.