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, 95% occur in children before the age of 5 years. The tumor may be in one eye or in both eyes. Retinoblastoma is usually confined to the eye and its nerve connections, and does not usually spread to other parts of the body.
Retinoblastoma may be hereditary (runs in families) or nonhereditary. The hereditary form may be in one or both eyes and generally affects younger children. Most retinoblastoma occurring in only one eye is not hereditary and is more often found in older children. When the disease occurs in both eyes, it is always hereditary. Because of the hereditary factor, brothers and sisters of children with retinoblastoma should have periodic examinations, including genetic counseling, to determine their risk for developing the disease.
A child with hereditary retinoblastoma has a higher incidence of a tumor in the brain, called trilateral retinoblastoma. MRI monitoring during and after treatment should occur. If your child has retinoblastoma, particularly the hereditary type, there is an increased chance that he or she may develop a second cancer in later years. It is therefore important to continue taking your child for medical check-ups even after the retinoblastoma has been treated.
Your child's prognosis (chance of recovery and of retaining sight) and choice of treatment depend on the extent of the disease within and outside of the eye. Most children with retinoblastoma survive.
Stages of retinoblastoma
Once retinoblastoma is diagnosed, 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 (within the eye) and extraocular disease (outside the eye).
Cancer is found in one or in both eyes but does not extend beyond the eye into the tissues around the eye or to other parts of the body. The five-year survival rate is greater than 90%.
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, typically the brain. The five-year survival rate is less than 10%.
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 or elsewhere in the body. The prognosis depends upon the site of recurrence and the extent of the recurrence.
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 consider both the attempt to cure and preservation of sight, and include the following:
Surgery to remove the eye especially if the tumor is large and/or there is little expectation for useful vision.
The use of extreme cold to destroy cancer cells.
The use of laser light to destroy blood vessels that feed the tumor, especially in small tumors.
The use of heat to destroy cancer cells.
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).
Chemotherapy is 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. Chemotherapy drugs may be taken by mouth or injected into a vein (intravenous) or a muscle. In children with retinoblastoma, chemotherapy drugs may be injected directly into the fluid that surrounds the brain and spinal cord (intrathecal chemotherapy).
Treatment by stage and clinical trials
Your child may receive treatment that is considered standard based on its effectiveness in patients in past clinical trials, or you may choose to have your child take part in a clinical trial. Clinical trials are designed to test potentially better treatments against those treatments currently considered standard of cure in hope of finding better ways to treat children with cancer. Clinical trials are ongoing in many parts of the country for advanced stages of retinoblastoma. The good news is that clinical trials are closely overseen by data monitoring committees, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.
- 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 or when there is no expectation that useful vision can be preserved.
- External radiation therapy, photocoagulation, cryotherapy, brachytherapy or chemotherapy 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 radiation therapy (with or without other types of treatment) 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 local control therapy.
Treatment may be one of the following:
Radiation therapy and/or intrathecal chemotherapy in patients with central nervous system disease. Clinical trials are also testing new combinations of chemotherapy drugs and new ways of administrating chemotherapy drugs. Radiation therapy and systemic chemotherapy is being evaluated as is the use of stem cell reconstitution.
Treatment depends on the site and extent of the recurrence (or progression). If the cancer comes back only in the eye, your child may have surgery or radiation therapy. If the cancer comes back outside of the eye, treatment depends on many factors and is based on individual patient concerns; you may want to have your child participate in a clinical trial.
Cleveland Clinic Children's
Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.
Should you have more questions about the child or young adult in your life with cancer, please contact Cleveland Clinic Cancer Answer line at 216.444.3024 or 800.223.2273, extension 4-3024. Your question will be forwarded to the Chair of the Pediatric Oncology Department.
We wish you and your child well.
For other websites regarding childhood cancer:
Source: National Institutes of Health, National Cancer Institute, Children's Oncology Group. Rev. 1/03