n WHAT IS STAGING? Staging is a very important process that is used to identify the location and size of the tumor at the time of diagnosis. For several decades, the Reese-Ellsworth staging system was used as the primary interna- tional staging system for retinoblastoma. It was designed to predict the chance of controlling the disease and saving the vision in the affected eye(s). In the late 1990s, a new staging system called the ABCDE system was developed, and it has replaced the Reese-Ellsworth system at most treatment centers. The ABCDE system grades tumors by their size, number, and location in the retina. The advantage of this new system is that it more accurately describes the degree of disease in the eye, helping the treating doctor(s) to better determine the type of therapy needed for each child. n WHICH TESTS AND PROCEDURES WILL MY CHILD NEED? The following tests may need to be done to evaluate the location and extent of the cancer and determine the most appropriate treatment. All children should receive a magnetic resonance imaging (MRI) or computed tomography (CT) scan of the brain and orbits (eyes), an eye examination under anes- thesia (EUA), and possibly an ultrasound of the orbits. Some children who have a higher-stage disease also will need a bone marrow aspirate, bone marrow biopsy, bone scan, and blood tests. Although advanced retinoblastoma is uncommon in the United States and other developed countries, these tests are still needed to ensure that the retinoblastoma has not spread to other parts of the body. MRI An MRI is a test that gives very exact pictures of the organs and tumors inside the body. During an MRI test, your child will lie on a table that moves into a tube-like machine surrounding him or her with a mag- netic field. The test is painless, but the machine makes quite a bit of noise. Your child must not move at all during the test; therefore, your child will be put to sleep with medicine (IV sedation or general anesthesia) so that he or she will be able to lie completely still during the test. For suspected retinoblastoma, an MRI of the brain and orbits (eyes) is performed. An MRI does not expose your child to any form of radiation. CT SCAN The CT scan is a computer-assisted X ray that shows very precise pictures of soft tissues, internal organs, and tumors. The scan is painless, but your child must be able to lie absolutely still during the scan (younger children may require sedation to put them to sleep). A small amount of contrast (dye) is injected into your child’s vein to show more detailed pictures. When your child receives the contrast into their vein (via an IV), he or she may have a warm, flushing feeling. Generally, there are no side effects from the con- trast, although a small number of children may experience an allergic reaction. Medications are available immediately if an allergic reaction occurs. EYE EXAM UNDER ANESTHESIA (EUA) Retinoblastoma is unlike other tumors because a biopsy of the tumor cannot be obtained by surgically removing a piece of it from the eye for examination. Instead, a thorough eye exam under anesthesia (EUA) is performed. During EUA, your child is put into a deep sleep, and the ophthalmologist (eye doctor)
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