Pediatric Hematology/Oncology

What is Retinoblastoma?

Retinoblastoma is a cancer that occurs as the result of abnormal cell growth in the retina of the eye. The retina is the thin nerve tissue that lines the back of the eye that senses light and forms images.

This type of cancer is very rare, and although it can occur at any age, it usually occurs in younger children, typically before the age of five. It can be hereditary or non-hereditary. Retinoblastomas that occur in only one eye are usually not hereditary, and retinoblastomas found in both eyes are always hereditary. The hereditary form usually develops before a child reaches age 2, while the non-hereditary form often develops later.

Risk Factors

Unlike cancers that develop among adult patients, typically there are no known risk factors for childhood cancers. Researchers have identified a few characteristics of retinoblastoma patients, but typically, there is nothing that a child or parent can do to prevent childhood cancers from developing.

  • Age. Average age of retinoblastoma patients is two years old. Eighty percent are diagnosed before age four. It is extremely rare in adults.
  • Heredity. About 40% of all cases are inherited, due to a genetic mutation passed down from one parent.
  • Race. Occurs equally in all races.
  • Gender. Occurs equally between boys and girls.

Common Symptoms

Retinoblastoma is usually diagnosed due to a parent or pediatrician noticing an abnormal appearance in a child's eye(s). The parent or pediatrician may notice the following:

  • Cat's eye reflex, or leucocoria: Usually, when a light is shone in someone's eye, the pupil will look red. A pupil that appears white is cause for concern.
  • Eyes looking in different directions: Although this symptom can indicate other, less serious, conditions, when a child's eyes appear to be looking in different directions, this needs to be checked out by a doctor.

Less common symptoms include:

  • Eye pain
  • Redness in the white of the eye
  • Pupil does not retract when exposed to bright light

Any of these symptoms can indicate retinoblastoma or other, less, serious conditions. It is important to have your child's eyes checked regularly, either by their pediatrician or an ophthamologist, especially if there have been other occurrences of retinoblastoma within the family.

Diagnosing Retinoblastoma

Typically, retinoblastoma can be diagnosed through an eye exam only, if it is performed by an ophthamologist (a medical doctor who specializes in treatment of the eyes) with experience with this disease. A pediatrician will perform an eye exam at birth, and then at least once a year. The doctor may give your child a general anesthesia, and then will exam the eye by using special lights and magnifying lenses to view the retina. If there is a family history of retinoblastoma, your child should be seen by an ophthalmologist. If the diagnosis is retinoblastoma, additional tests may be performed to determine the stage (progress) of the disease.

Treatment Options

Once the diagnosis is confirmed, your child's doctor will work with you and your child to determine the best treatment plan. Treatment options will depend upon the location and stage of the cancer, as well as your child's age and general health. Current treatment options may include surgery, radiation therapy and/or chemotherapy.

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Pediatric Hematology/
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Golisano Children's Hospital
601 Elmwood Avenue
Rochester, NY 14642
Phone: (585) 275-2981
Fax: (585) 273-1039

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