Pediatric Oncology Program

Patient and Family Education

Retinoblastoma

What Is Retinoblastoma?

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Retinoblastoma is a cancerous tumor of the retina, the thin nerve tissue lining the back of the eye which senses light and forms images.The disease occurs most commonly in younger children, usually before the age of five. About 300 children are diagnosed with retinoblastoma each year in the United States. The disease accounts for about 3 percent of childhood cancers. Adults can develop retinoblastoma too, but it is extremely rare.

Retinoblastoma may be hereditary or nonhereditary. The hereditary form generally affects younger children and may occur in one or both eyes. A child who has tumors in both eyes almost always has the hereditary form of the disease. Retinoblastoma that occurs in only one eye usually is the nonhereditary form, which is more often found in older children. About 75 percent of cases occur in only one eye, and about 25 percent occur in both eyes.

Usually retinoblastoma is confined to the eyes and does not spread to other organs or tissues. If it is found early, it is treatable and the child's eyesight can often be saved. In rare cases it can spread to the brain or bone marrow and becomes difficult or impossible to treat.

If a child has retinoblastoma, particularly the hereditary type, there is an increased chance that he or she may develop a second cancer in later years. Parents should therefore continue taking their child for regular medical checkups after the cancer has been treated.

A child's prognosis (chance of recovery and of retaining sight) and the choice of treatment for retinoblastoma depend on the extent of the disease within and beyond the eye.

This page was last updated on: October 20, 2009.