Retinoblastoma
What Is It?
Retinoblastoma, a cancerous tumor of the retina in the eye, is a rare form of cancer that affects children, most commonly before age 5. The retina is the nerve tissue at the back of the eye that senses light and sends images to the brain, which interprets these images.
The disease affects about one in every 15,000 to 20,000 children. According to the American Cancer Society, retinoblastoma is responsible for 5% of childhood blindness. However, with treatment, 85% of children with retinoblastoma maintain their sight.
Symptoms
The most common sign of retinoblastoma is an unusual whitish appearance of the pupil (a condition known as leukocoria). This condition is seen frequently in children with retinoblastoma, but it does not always indicate retinoblastoma. Children with retinoblastoma also may have a crossed eye (strabismus) that turns out toward the ear or in toward the nose. Strabismus, however, is a common condition and more likely to be noncancerous (benign). About 10% of children with retinoblastoma develop a persistent red irritation and inflammation of the eye. Less common symptoms include differences in iris color and pupil size, tearing, cataract or bulging of the eyes.
Prevention
Because retinoblastoma may be hereditary, genetic counseling and testing is an important part of disease management. The American Cancer Society reports that patients who carry the gene for the disease have about an 80% chance of developing retinoblastoma and a 50% chance of passing the gene to a child. Therefore, all siblings and children of retinoblastoma patients should be examined every two to four months during the first years of life.
Treatment
The type of treatment for retinoblastoma will depend on whether the disease is in one or both eyes, the extent of the disease within the eye, whether vision can be saved, and whether the cancer has spread beyond the eye. This is a rare disease, and treatment should be sought from centers of expertise with experience treating this type of cancer.
If the tumor is large, in one eye, and vision cannot be preserved, surgery may be done to remove the whole eye (enucleation). This is a simple operation, and a child usually can be fitted with an artificial eye within about three to six weeks following surgery.
