Following treatment for retinoblastoma, the main concerns for most families are the immediate and long-term effects of the tumor and its treatment, and concerns about possible recurrence of the tumor.

It is certainly understandable to want to put the tumor and its treatment behind you and to get back to a "normal" life. But it's important to realize that follow-up care is a central part of this process that offers your child the best chance for recovery and long-term survival.

Follow-up exams

Once treatment is finished, the health care team will discuss a follow-up schedule with you, including which tests should be done and how often. Doctor visits and tests to look for signs of recurrence are done more frequently at first. If nothing abnormal is found, the time between tests can then be extended.

If a child with retinoblastoma in only one eye has been treated by enucleation (removal of the eye), regular exams are needed to look for tumor recurrence, metastases, or any growth irregularities related to surgery. It is also important to have the remaining eye checked regularly so that if a second retinoblastoma develops later on it can be found and treated as early as possible.

For children treated with radiation therapy, laser therapy, cryotherapy, or treatment other than removal of the eye, close follow-up exams by an ophthalmologist are very important. In children with hereditary retinoblastoma, it is very common for new tumors to form until they are 3 or 4 years old. This is not a failure of the treatment, but the natural process in bilateral retinoblastoma. Therefore, it is very important that even after completing all treatments, children are examined regularly by specialists. General anesthesia may be needed to keep a young child still enough for the doctor to do a thorough exam. This is done to be certain the cancer has been completely destroyed, to find recurrences as early as possible, and to find problems with vision caused by treatments.

It is important for you to report any new symptoms your child is having, such as pain or vision problems, to your doctor right away, since they could be an early warning of cancer coming back or long-term side effects of treatment.

Genetic counseling and testing

If you have a child diagnosed with retinoblastoma, your family may be referred for genetic counseling. This is because some cases of retinoblastoma are caused by a genetic mutation that can be inherited.

If a child is diagnosed with retinoblastoma in both eyes, it can be assumed that they have the hereditary form of the disease, which means they carry the mutant Rb gene in all their cells. It's also possible that children with retinoblastoma in only one eye carry the mutant Rb gene in all their cells. This can be confirmed with a blood test. Children with this mutant gene are at increased risk for developing cancer later in life (see the section "Second cancers" below).

If the child carries the mutated Rb gene, then other children in the family may have inherited the same abnormal gene as well, and are at risk of being affected. Meeting with a genetic counselor can give you a better idea of what this risk might be and if other children in the family should be tested for the mutation. The genetic counselor will:

• Review the child's medical records and ask questions about other relatives to estimate the likelihood of an inherited gene affecting some family members.

• Provide information and answer questions about genetic testing, and schedule tests for other children in your family (if needed) so that their risk of developing retinoblastoma can be determined.

If tests show your children are at risk of developing retinoblastoma, their doctors will follow them very closely to find retinoblastoma at the earliest possible stage, if it occurs. It is very helpful to be able to tell which children have inherited the mutation that leads to retinoblastoma, since those children will need to be monitored closely.

In some cases it is not possible to tell with certainty if a child inherited the Rb gene mutation. In those cases the safest plan is to monitor children in the family closely for retinoblastoma with frequent eye exams.

Keeping good medical records

As much as you may want to put the experience behind you once treatment is completed, it is also very important to keep good records of your child's medical care during this time. This can be very helpful for your child later on as an adult and his or her doctors. Be sure your child's doctors have the following information:

• a copy of the pathology report from any biopsies or surgeries
• if there was surgery, a copy of the operative report
• if there were hospitalizations, copies of the discharge summaries that doctors prepare when patients are sent home
• if there was chemotherapy treatment for the cancer, a list of the drugs, drug doses, and when they were given
• if there was radiation, a summary of the type and dose of radiation and when and where it was given

Long-term effects of cancer treatment

With major advances in treatment in recent decades, many children treated for retinoblastoma are now surviving into adulthood. Because childhood cancer survivors are living longer, their health in adulthood has become more of a concern in recent years. Researchers have learned that treatment may affect that child's health later in life.

Just as the treatment of childhood cancer requires a very specialized approach, so does the care and follow-up after treatment. The earlier any problems can be recognized, the more likely it is they can be treated effectively.

The risk of late effects depends on a number of factors, such as the specific treatments the child received, the doses of treatment, and the age of the child when being treated. These late effects may include:

• reduced kidney function
• heart or lung problems after receiving certain chemotherapy drugs or radiation therapy to these parts of the body
• slowed or decreased growth and development
• changes in sexual development and ability to have children
• development of other cancers (see next section)

To help increase awareness of late effects and improve follow-up care of childhood cancer survivors throughout their lives, the Children's Oncology Group (COG) has developed long-term follow-up guidelines for survivors of childhood cancers. These guidelines, written for doctors and other health care professionals, describe in detail the suggested long-term follow-up care based on the treatments the child has received. It is very important to discuss possible long-term complications with your child's health care team, and to make sure there is a plan in place to watch for these problems and treat them, if needed. To learn more, ask your child's doctors about the COG survivor guidelines, and see the separate American Cancer Society document, Childhood Cancer: Late Effects of Cancer Treatment.

Second cancers

Survivors of the hereditary form of retinoblastoma have an increased risk for developing other types of cancer throughout their lives. This is because each cell in the body has an abnormal Rb tumor suppressor gene, which would normally help stop some of these cancers from forming. A child who carries the mutant Rb gene has a much higher risk of developing another cancer sometime in their life. Most of these cancers are very treatable if detected early, which is why it is very important that these children are followed closely throughout life. The entire body must be carefully examined to avoid missing these second cancers.

The most common secondary cancers among retinoblastoma survivors include:

• osteosarcoma (a type of bone cancer)
• soft tissue sarcomas (cancers that develop in muscle, tendons and ligaments, and fatty tissue)
• melanoma (a type of skin cancer)
• brain tumors
• lung cancer
• lymphoma
• breast cancer

The risk for these cancers is increased if any of these areas received radiation during radiation therapy for retinoblastoma. Younger children treated with radiation therapy are more likely than older children to develop side effects such as second cancers or problems with bone growth in the irradiated area. Chemotherapy with certain drugs can also increase the risk of some cancers.

Because of the increased risk these children face, it's important that they're taught about other factors that might increase their risk of cancer as they get older. For example, sun exposure will increase the melanoma risk even further, and smoking will increase lung cancer risk.

Children with hereditary retinoblastoma also have a small risk of developing a tumor in the pineal gland within a few years. (This is known as trilateral retinoblastoma.) The pineal gland is a bean-sized structure lying under the middle of the brain. It can have cells similar to retina cells, which is why tumors can start there. For this reason, doctors often recommend that MRI scans be done regularly for 3 or 4 years to try to detect such tumors as early as possible.

Psychosocial issues for retinoblastoma survivors and their families

Most cases of retinoblastoma develop during a very sensitive time in a child's life. The effect will be greatest during the first year of treatment. The treatment center should evaluate the patient's family situation as soon as possible. If the patient or family members have concerns, they can be addressed before they become a crisis.

Centers that treat many patients with retinoblastoma may have programs to introduce new patients and their families to others who have finished their treatment. Seeing another patient with retinoblastoma doing well is often the best inspiration for a patient and family.

If needed, centers can also refer patients to special programs and facilities for the visually impaired. Most patients treated for retinoblastoma in only one eye will have normal vision in the unaffected eye, but they may have a cosmetic deformity in the treated eye. The cosmetic problems can often be lessened by treatment in a center with expertise in reconstructive surgery. Early intervention and counseling can also help address any psychological effects of changes in appearance.

Last Medical Review: 10/26/2009
Last Revised: 10/26/2009