• Retinoblastoma Overview

    Retinoblastoma is a malignant tumor in the retina, the light-detecting cells at the back of the eye. Thanks to advances in diagnosis and treatment, virtually all children with retinoblastoma now survive well into adulthood.

    Retinoblastoma Treatment at Dana-Farber/Boston Children's

    Children with retinoblastoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Retinoblastoma Program. Continue reading to learn more about retinoblastoma or visit the Retinoblastoma Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    These tumors are typically diagnosed in children less than 5 years of age, with the majority of cases diagnosed before age 2. Nearly 75 percent of the time, only one eye is affected. When retinoblastoma is inherited from a parent (through a mutated gene), there’s a chance it may develop later in the other eye. While rare, the disease can spread to other parts of the body, especially to the bone marrow.

    Symptoms tend to develop between ages 1 and 2 and include:

    • Leukocoria – pupil appears white instead of red when light shines into it; the most common symptom, but not required for diagnosis
    • Strabismus or “wandering eye” – one or both eyes do not appear to be looking in the same direction
    • Pain or redness around the eye(s)
    • Poor vision or change in vision

    In addition to a complete physical examination, doctors diagnose retinoblastoma with:

    • Complete fundoscopic (eye) exam – under anesthesia, the child’s pupils are dilated so the doctor can examine inside the eye, including the retina and optic nerve
    • Computerized tomography (CT or CAT) scan
    • Magnetic resonance imaging (MRI)
    • Ultrasound of the eye

    In some cases, genetic testing is done to look for the retinoblastoma gene, confirm the diagnosis, or determine if the genetic mutation is inherited. Biopsies are not usually done. After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Because of the risk that the eye can be damaged during cancer treatment, our retinoblastoma treatment team will utilize intra-arterial chemotherapy when possible. Intra-arterial chemotherapy is a newer treatment for more advanced retinoblastoma in which the medicine is injected directly into the main blood vessel of the eye (ophthalmic artery). This treatment was designed to minimize the amount of contact the chemotherapy has with the rest of your child’s body, and reduce side effects. It can deliver a much higher dose of chemo straight to the eye, and may kill more cancer cells in fewer treatments.

    During this procedure, your child is put to sleep by an anesthesiologist. Then the surgeon inserts a thin tube through a blood vessel in your child’s groin, and feeds it up to the ophthalmic artery. Then the medication is injected through the tube up to the eye.

    The average number of treatment sessions is about three for each eye, delivered every four weeks.

    Other treatment options include:

    • Chemotherapy – until recently, chemotherapy was used to treat retinoblastoma that had spread outside the eye, but now doctors use it as a first-line treatment. 
    • Surgery – an enucleation procedure to remove the diseased eye and replace it with an orbital implant and then an artificial eye after the surgery has healed; usually reserved for advanced cases that do not respond to other therapies
    • Cryotherapy – applying extreme cold directly to the tumor
    • Thermotherapy – delivering heat to the tumor
    • Photocoagulation – the use of infrared light from a laser to destroy the tumor’s blood supply
    • Radiation therapy 

    Progressive or Recurrent Disease

    Retinoblastoma that recurs after surgery is treated with aggressive chemotherapy and local radiation therapy. If the recurrence is outside of the eye, a clinical trial may be considered.

    Long-term Outlook

    One hundred years ago, retinoblastoma was almost always fatal. Today, retinoblastoma has one of the highest cure rates of all childhood cancers—about 95 percent to 98 percent—with more than nine out of every 10 children surviving well into adulthood. Lifelong follow-up is crucial because children who have had retinoblastoma are at risk of developing cancer elsewhere in the body later in life.
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  • Treating Retinoblastoma

    Intra-Arterial Chemo Brochure 

    Our doctors are using intra-arterial chemotherapy for retinoblastoma, a treatment designed to minimize the amount of chemotherapy and reduce side effects. It can deliver a much higher dose of chemo straight to the eye, and may kill more cancer cells in fewer treatments.