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About a Retinoblastoma Tumor

Retinoblastomas form on the retina (the nerve tissue lining the inside of the back of the eye) of one or both eyes. These cancerous tumors occur almost exclusively in young children who have a genetic predisposition, but they usually do not spread to other tissues, are easily removed and rarely prove fatal.

  1. Causes

    • Children who have at least one parent who lacks a portion of the long arm of chromosome 13 have a 50 percent chance of developing a retinoblastoma. Some retinoblastomas appear in children without this inherited risk for the condition, and it is unknown why those cases occur.

    Symptoms and Diagnosis

    • Retinoblastomas can make it difficult to focus both eyes in the same direction and may cause the irises of the eyes to turn white. The tumors can also cause pain and redness in the eyes. Eye doctors may spot larger retinoblastomas simply by shining a light into the eyes; the tumors show up well on CT and MRI scans.

    Complications

    • Undetected and untreated retinoblastomas can cause blindness and, in a handful of cases, spread to the pineal glands near the center of the brain, creating a usually fatal condition called trilateral retinoblastoma. Having had a retinoblastoma also raises a person's risk for developing other cancers later in life.

    Treatment

    • Large retinoblastomas may require the removal of the affected eye or eyes. For people who have smaller tumors---meaning their vision can be saved with appropriate treatment---therapeutic options include freezing or burning the tumor or its blood vessels, cutting the tumor out with a laser, chemotherapy and radiation therapy.

    Prevalence

    • Retinoblastomas usually occur in children younger than five years of age, with only 5 percent of these tumors being found in older individuals. The tumors appear rarely, however. Only about 300 U.S. children develop retinoblastomas each year.

    Prognosis

    • Among all children with retinoblastomas diagnosed between 1976 and 1994, 93 percent survived for at least five years following their diagnosis. At least 20 percent of patients did lose some or all vision in at least one eye.

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