Retinoblastoma

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Retinoblastoma (reh-tin-oh-blas-TOE-mah) is a malignant (cancer) tumor of the eye. The tumor is in the retina which is in the back of the eye. Retinoblastoma is rare and is most often found in children younger than 2 years of age. It is very rare for the tumor to appear in people over 6 years of age.

There are two types of retinoblastoma:

  • Sporadic: 60% of retinoblastoma cases are sporadic (occur by chance). Most unilateral (one eye) retinoblastoma are by chance.
  • Inherited: When retinoblastoma occurs in both eyes, there is an inherited factor. This means it is passed from parent to child. Siblings of the affected child should have regular exams by an ophthalmologist (eye doctor).

Healthy parents who have one child with the disease have a 6% chance that another child may also have it.

Signs and Symptoms

Retinoblastoma is hard to diagnose early because symptoms may not be obvious. Children with retinoblastoma often:

  • Have a new onset of crossed eyes.
  • Squint or have trouble focusing both eyes on the same object.
  • Have a white pupil. This can be seen by parents or in pictures. It is also called cat-eye reflex.

Symptoms that occur in later stages include:

  • red, painful eye
  • a decrease or loss of sight in the affected eye

Diagnosis

A child with any of these signs or symptoms needs to go to an ophthalmologist. The eye doctor will perform:

  • A complete dilated eye exam. They may also do an eye exam under general anesthesia in the operating room.
  • An MRI (magnetic resonance imaging) to see if a tumor is outside the eye or in the brain.

If your child’s health care provider thinks the disease has spread to other parts of their body, a bone marrow test, bone scan and spinal tap may be done.

Treatment

The size, location and how the tumor has spread within the eye, all make a difference in your child’s treatment. Possible treatments for retinoblastoma include:

  • chemotherapy
  • laser therapy
  • radiation therapy
  • cryotherapy
  • Enucleation (removal of the eye) – if other treatments fail or the eye is blind and painful.

Several of these treatments may be combined. The ophthalmologist and oncologist (cancer doctor) will talk with you about the best treatment plan for your child.

Follow-up Care

  • Your child will have follow-up appointments with an oncologist and an ophthalmologist.
  • Because retinoblastoma is sometimes inherited, your family is encouraged to get genetic counseling to learn more about the disease.
  • Exams and MRIs will be done occasionally to follow your child’s response to treatments.
  • If needed, your child will be fitted for a prosthesis (artificial eye).

If you have any questions or concerns, be sure to talk with your child’s health care provider.

Retinoblastoma (PDF)

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