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Retinoblastoma Overview

written by: R. Elizabeth C. Kitchen • edited by: Emma Lloyd • updated: 9/23/2010

Retinoblastoma is a tumor of the retina. Read on for a comprehensive overview of this cancer.

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    Retinoblastoma is a rare type of cancer affecting the retina. Young children are most often affected by this cancer, while it rarely affects adults. Though this eye cancer is rare, it is the most common eye cancer affecting children. This cancer can occur in one eye or both eyes.

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    Symptoms and Complications

    The signs and symptoms of this cancer are the same for all ages. They include:

    • The eyes seeming to be looking in different directions
    • Eye swelling
    • When light is focused on the eye, a white color appears in the pupil
    • Eye redness

    One of the main complications is this cancer recurring. Children who have the inherited form are at an increased risk of developing other types of cancers (not just limited to the eye) in the future.

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    Causes

    Genetic mutations affecting the retina's nerve cells can cause the cells to grow and multiply, resulting in a tumor. This cancer can also spread to the spine, brain, and other areas of the body. In most cases, what caused the genetic mutation that results in this cancer is unknown. However, it is known that children may inherit this genetic mutation.

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    Diagnosing this Cancer

    An eye exam performed by an ophthalmologist is often the first test performed. In some cases, the patient will be anesthetized and a more thorough eye exam will be performed.

    Diagnostic imaging tests are also often done. These can be done to determine whether or not this cancer has spread, or is also affecting other structures surrounding the eye. Commonly performed imaging tests include ultrasound, magnetic resonance imaging, and computerized tomography.

    Once testing has been completed, the patient's ophthalmologist may also consult with other specialists to determine if further testing is needed, or to create a treatment method. Such specialists include oncologists, a surgeon, or a genetic counselor.

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    Treatment

    Treatment will ultimately depend on the tumor location and size, and whether retinoblastoma has spread. Whenever possible, the patient's doctor will do what she can to preserve the patient's vision.

    Chemotherapy is often the first line of treatment. This may help to shrink the tumor, allowing other treatments more effective in completely eradicating the tumor. External beam radiation or internal radiation is often done in combination with chemotherapy to help treat this eye cancer by killing cancer cells.

    Laser therapy can be done to help in destroying the tumor's blood vessels. This results in tumor death because without blood vessels, the tumor will not receive vital nutrients and oxygen.

    Cryotherapy kills cancer cells using extreme cold. Heat therapy may also be used and it uses extreme heat to destroy cancer cells.

    Surgery is often performed when other methods have failed or when the tumor is too large. Commonly performed surgical procedures include an eye implant, removing the affected eye, or to fit an artificial eye.

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    Resources

    MayoClinic.com. (2010). Retinoblastoma. Retrieved on September 12, 2010 from MayoClinic.com: http://www.mayoclinic.com/health/retinoblastoma/DS00786

    MedlinePlus. (2010). Retinoblastoma. Retrieved on September 12, 2010 from MedlinePlus: http://www.nlm.nih.gov/medlineplus/ency/article/001030.htm