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Retinoblastoma and Your Child's Eyes

Retinoblastoma is a malignant tumor on the retina, the light-sensing part of the eye, and is highly curable if treated early. This type of cancer can be present in one or both eyes.

What Causes Retinoblastoma?

Eyes begin to develop very early in the womb. Rapidly growing cells in the eye, called retinoblasts, will eventually mature and form the retina, the light-sensing part of the eye that is located in the back of the eye. Sometimes, these specialized cells do not stop reproducing and form a tumor on the retina. These tumors may continue to grow, filling almost the entire vitreous humor (the jellylike substances the fills the eyeball). These tumors can also break off and spread to other parts of the eye and eventually outside to lymph nodes and other organs.

Who Gets Retinoblastoma?

Retinoblastoma occurs most often in children 5 years and under. It rarely occurs in adults. Between 200 and 300 children are diagnosed with retinoblastoma each year, affecting one in every 20,000. About 40% of all cases of retinoblastoma are inherited, meaning the defective cancer gene is passed on from parent to child. Retinoblastoma occurs about 75% of the time in one eye, and 25% of the time in both eyes. Inherited retinoblastoma is more likely to occur in both eyes.

What Are the Symptoms of Retinoblastoma?

Symptoms of retinoblastoma include:

  • A pupil that appears white when light is shone into it. This may mean that a retinal tumor is present. Blood vessels in the back of the eye will normally reflect red.
  • The child's pupil does not have the normal red appearance that a flash photo creates. Instead, the pupil is white.
  • Eyes that may not move or focus in the same direction.
  • Eye pain.
  • A pupil that is constantly dilated.
  • Red eye or eyes.

How Is Retinoblastoma Diagnosed?

An eye exam and imaging test given by an eye doctor can diagnose retinoblastoma.

If retinoblastoma is suspected, an ophthalmologist -- an MD who specializes in treating eyes -- will need to examine the eye using special equipment to see the retina. Other tests may be conducted to determine the stage of the tumor, or how far it has spread. These tests include ultrasound, MRI scans, CT scans, bone scans, spinal tap, and bone marrow tests.

What Are the Stages of Retinoblastoma?

Stages of retinoblastoma include:

  • Intraocular retinoblastoma. The earliest stage of retinoblastoma, found in one or both eyes. It has not yet spread to tissue outside the eye.
  • Extraocular retinoblastoma. This type of cancer has either spread outside of the eye or to other parts of the body.
  • Recurrent retinoblastoma. The cancer has come back or spread in the eye or to other parts of the body after being treated.

WebMD Medical Reference

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