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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.
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.
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.
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 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.
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:
Surgery to remove the eye.
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).
The use of extreme cold to destroy cancer cells.
The use of laser light to destroy blood vessels that supply nutrients to the tumor.
The use of heat to destroy cancer cells.
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. Clinical trials are ongoing in many parts of the country for advanced stages of retinoblastoma. For more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.
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.
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.
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.
Changes to This Summary (08/19/2004)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
To Learn More
For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.
Web sites and Organizations
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The NCI's LiveHelp service, a program available on several of the Institute's Web sites, provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
For more information from the NCI, please write to this address:
NCI Public Inquiries Office
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.
Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.
Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.