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Retinoblastoma

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What is retinoblastoma?

Retinoblastoma is a malignant (cancerous) tumor of the retina. The retina is the thin nerve tissue that lines the back of the eye that senses light and forms images.

Although retinoblastoma may occur at any age, it most often occurs in younger children, usually before the age of 5 years. The tumor may be in one eye only or in both eyes. Retinoblastoma is usually confined to the eye and does not spread to nearby tissue or other parts of the body. Your child’s prognosis (chance of recovery and retaining sight) and choice of treatment depend on the extent of the disease within and beyond the eye.

This disease is caused by mutations in a gene called the Retinoblastoma-1 (RB1) gene. These mutations are either inherited (passed from the parents to the children) or new (not passed from the parents to the children) mutations. Some new mutations may become "inherited" (the new mutation is passed from the parents to the children). Tumors caused by inherited mutations are called hereditary retinoblastomas. Tumors caused by new mutations are called sporadic retinoblastomas. Hereditary retinoblastomas may form in one or both eyes, and they are generally found in younger children. Most retinoblastomas that form in only one eye are not hereditary, and they are found more often in older children. When tumors form in both eyes, the disease is almost always hereditary. Because of the hereditary factor, patients and their brothers and sisters should have periodic examinations, including genetic counseling, to determine their risk for developing the disease.

A child who has hereditary retinoblastoma may also be at risk of developing a tumor in the brain while they are being treated for the eye tumor. This is called trilateral retinoblastoma, and patients should be periodically monitored by the doctor for the possible development of this rare condition during and after treatment. If your child has retinoblastoma, particularly the hereditary type, there is also an increased chance that he or she may develop other types of cancer in later years. Parents may therefore decide to continue taking their child for medical check-ups even after the cancer has been treated.

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Stage Explanation

Stages of retinoblastoma

Once retinoblastoma is found, more tests will be done to determine the size of the tumor and whether it has spread to surrounding tissue or to other parts of the body. This is called staging.

To plan treatment, your child’s doctor needs to know the stage of disease. Although there are several staging systems currently available for retinoblastoma, for the purposes of treatment retinoblastoma is categorized into intraocular and extraocular disease.

Intraocular retinoblastoma

Cancer is found in one or both eyes, but does not extend beyond the eye into the tissues around the eye or to other parts of the body.

Extraocular retinoblastoma

The cancer has extended beyond the eye. It may be confined to the tissues around the eye, or it may have spread to other parts of the body.

Recurrent retinoblastoma

Recurrent disease means that the cancer has come back (recurred) or progressed (continued to grow) after it has been treated. It may recur in the eye, the tissues around the eye, or elsewhere in the body.

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Treatment Option Overview

How retinoblastoma is treated

There are treatments for all children with retinoblastoma, and most children can be cured. The type of treatment given depends on the extent of the disease within the eye, whether the disease is in one or both eyes, and whether the disease has spread beyond the eye. Treatment options that attempt to cure the patient and preserve vision, include the following:

  • Enucleation: Surgery to remove the eye.
  • Radiation therapy: Radiation therapy uses high-energy radiation from x-rays and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy) or may be administered by placing radioactive material into or very near the tumor (internal radiation therapy or brachytherapy).
  • Cryotherapy: The use of extreme cold to destroy cancer cells.
  • Photocoagulation: The use of laser light to destroy blood vessels that supply nutrients to the tumor.
  • Thermotherapy: The use of heat to destroy cancer cells.
  • Chemotherapy: The use of drugs to kill cancer cells. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body. In children with retinoblastoma, chemotherapy is under investigation.

Treatment by stage

Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child take part in a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and find better ways to treat children with cancer.

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Intraocular Retinoblastoma

Treatment depends on whether the cancer is in one or both eyes.

If the cancer is in one eye, treatment may be one of the following:

  • Surgery to remove the eye (enucleation) is used for large tumors when there is no expectation that useful vision can be preserved.
  • External radiation therapy, photocoagulation, cryotherapy, thermotherapy, or brachytherapy may be used with smaller tumors when there is potential for preservation of sight.

If the cancer is in both eyes, treatment may be one of the following:

  • Surgery to remove the eye with the most cancer, and/or radiation therapy to the other eye.
  • Radiation therapy to both eyes if there is potential for vision in both eyes.
  • Clinical trials testing systemic chemotherapy with or without other types of treatment.

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Extraocular Retinoblastoma

Treatment may be one of the following:

  • Radiation therapy and/or intrathecal (into the space between the lining of the spinal cord and the brain) chemotherapy.
  • Clinical trials are testing new combinations of chemotherapy drugs, with or without peripheral stem cell transplantation, and different ways of administrating chemotherapy drugs.

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Recurrent Retinoblastoma

Treatment depends on the site and extent of the recurrence (or progression). If the cancer comes back only in the eye and is small, your child may have surgery or radiation therapy. If the cancer comes back outside of the eye, treatment will depend on many factors and individual patient needs. You may want to consider having your child participate in a clinical trial.

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Retinoblastoma