What is retinoblastoma?

Retinoblastoma is a tumor that arises from the retina. It usually affects children less than five years of age and the average age of diagnosis is 12 to 18 months. This condition affects approximately 300 children per year in the United States. With current modalities of treatment, the survival from retinoblastoma is more than 95 percent. However, early detection and intervention is very important to prevent spread to the brain and the rest of the body.

Anatomy of the eye

Retina is a thin membranous lining of the rear 2/3rd of the eye. It is the light sensitive tissue of the eye that converts images to electrical signals that are carried via the optic nerve.

What causes retinoblastoma?

The tumor develops due to a genetic abnormality in one of the chromosomes of the body. Ten percent of children who get retinoblastoma have a family history and 90 percent develop without any family history. Retinoblastoma is not caused by any environmental factors, and there is nothing that parents did or did not do that causes the gene abnormality.

What to look for

Most commonly, retinoblastoma is detected by the presence of a white glow in the pupil. It is often noticed by parents when they are reviewing photos of their children. Retinoblastoma may also present as eye drifting or crossing (strabismus).

Forty percent of children with Retinoblastoma have a genetic form of the disease, even if no one in their family has the disease. These children are at risk of developing retinoblastoma in both eyes. Your doctor will closely watch both eyes during the treatment course.