Retinoblastoma

Retinoblastoma is a rare type of eye cancer that develops in the retina, the part of the eye that detects light and color. Although this disorder can occur at any age, it usually develops in young children. Most cases of retinoblastoma occur in only one eye, but both eyes can be affected.

Symptoms

The most common sign of retinoblastoma is a change in the color of the pupil. The normally black pupil of the eye appears white, especially under bright light. This is sometimes referred to as a cat’s eye reflex.

It is common to see “red eye” in photographs that were taken using a flash camera. When the flash of a camera or another bright light produces the appearance of a white pupil, especially in one eye only, this is a sign of a serious underlying problem with the eye, and an eye doctor should be consulted immediately.

Sometimes an eye with retinoblastoma will cross or turn outward.

Treatment

With early diagnosis, retinoblastoma treatment is remarkably effective.  More than 90% of children survive and many eyes are saved with a combination of medications, radiation therapy, and heat, freezing, or laser treatments.

In severe cases, the affected eye is removed to avoid spread of the tumor.

Tests/Diagnosis

Parents are frequently the first to notice a white pupil when looking at photographs of their children. And primary care physicians may notice a pupil that appears white or an eye that isn’t straight when conducting a regular well-baby examination.

In addition to retinoblastoma, a white pupil is a sign of several diseases and conditions of infancy and childhood, including:

  • cataract

  • persistent hyperplastic primary vitreous (PHPV)

  • retinopathy of prematurity

  • toxocariasis

Causes/Risk Factors

Retinoblastoma can be hereditary and is more likely to develop in children with a family history of the disease.

Retinoblastoma can occur in one or both eyes, and usually develops in the first year or two of life. It affects children of all races, and occurs in boys and girls equally.

If a child has had retinoblastoma, there is an increased chance for a second cancer to develop elsewhere in the body. Children with retinoblastoma should have regular examinations by an eye doctor and a pediatric cancer specialist.