Retinoblastoma is a rare cancer of the retina of the eye. The retina is in the back of the eye. It’s the part of the eye that receives light. Retinoblastoma is the most common tumor affecting the eye in children. It almost always occurs in children less than 5 years old.
The cancer is caused by a change in a gene. It may be a gene passed down from parents (inherited). Or it may be a gene change that happens by chance (sporadic).
In 1 out of 3 children with retinoblastoma, it’s present at birth (congenital), and the gene change is int all cells in the body. This is known as hereditary retinoblastoma. Of these children, 1 out of 4 has the gene passed on from parents. In this form, the retinoblastoma usually affects both eyes. It also increases the risk of other cancers such as sarcoma and melanoma.
In 2 out of 3 children with retinoblastoma, it occurs by chance. In these cases, it affects only one eye. There is not an increased risk of other cancers.
Symptoms may affect one or both eyes. Symptoms can occur a bit differently in each child. They can include:
Less common symptoms may include:
Most of the time, retinoblastoma has not spread (metastasized) to other areas of the body when it’s diagnosed. If it has spread, other symptoms may include:
The symptoms of retinoblastoma can be like other health conditions. Make sure your child sees a healthcare provider for a diagnosis.
Your child's healthcare provider will ask about your child's health history and symptoms. He or she will examine your child and pay close attention to the eyes. The healthcare provider will likely advise that your child see an eye specialist (ophthalmologist).
The ophthalmologist will check your child's eyes with special tools. Your child may need a dilated indirect ophthalmoscopic exam. Your child may be given anesthesia medicine to sleep through the exam.
Your child may need other tests such as:
A child may be diagnosed with no symptoms. This may be the case if you have a family history of retinoblastoma. Your child may have eye exams often to check for signs of the cancer.
After a diagnosis of retinoblastoma, these tests will help your child's doctor know if the cancer is inside the eye, how much of the eye is involved, and if it has spread beyond the eye. This process is called staging. The stage of a cancer is one of the most important things to know when deciding how to treat it. Doctors use 2 standard staging systems for retinoblastoma.
The newer system is called the International Classification for Intraocular Retinoblastoma. It divides these tumors into 5 groups lettered A through E. The groups are based on how likely it is that the eye can be saved. Group A means the tumor is small and not near important structures that allow your child to see. These tumors can likely be removed and vision can be saved. The higher the group, the more advanced the tumor is. A tumor in Group E means that doctors have little to no chance of saving the eye. The other groups then fall between these extremes.
The Reese-Ellsworth system is older and less commonly used. It groups retinoblastomas much like the International Classification system, based on whether the eye and vision can be saved. The system uses groups numbered 1 through 5. Group 1 means a good chance of saving the eye, and Group 5 means it's not likely. The other groups fall between this.
Doctors may use other staging systems. The staging process for retinoblastoma can be very complex. Be sure to ask your child's healthcare provider to explain the stage of your child's cancer to you in a way you can understand.
Most children in the U.S. with retinoblastoma have very good prognoses. This means they have a good chance of recovery.
Your child will be treated by a team of different types of healthcare providers, such as:
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. It also depends on whether the retinoblastoma is hereditary.
Many of the treatment methods require sleep medicine (anesthesia). Treatment may include the following:
With any cancer, how well a child is expected to recover (prognosis) varies. Keep in mind:
A child may have complications from the cancer or from treatment, such as:
You can help your child manage his or her treatment in many ways. For example:
A child with retinoblastoma needs ongoing care. Your child will be cared for by a team of healthcare providers. They will treat any late effects of treatment and to watch for signs or symptoms of the tumor growing back. The healthcare providers will also check your child with imaging tests and other tests.
Note: If your child has an implant and artificial eye, he or she should wear protective eyewear during activities that may harm the eye.
Family members may want to see a genetic counselor to learn more about the risk for retinoblastoma.
Call the healthcare provider if your child has:
Tips to help you get the most from a visit to your child’s healthcare provider:
The University of Chicago Medicine
5841 S. Maryland Avenue
Chicago, IL 60637 | 773-702-1000
Appointments: Call UCM Connect at 1-888-824-0200