Retinoblastoma - Childhood

ON THIS PAGE: You will find information about the estimated number of children who will be diagnosed with retinoblastoma each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors, and no 2 people with cancer are the same. Use the menu to see other pages.

Every person is different, with different factors influencing their risk of being diagnosed with this cancer and the chance of recovery after a diagnosis. It is important to talk with your doctor about any questions you have around the general statistics provided below and what they may mean for your child individually. The original sources for these statistics are provided at the bottom of this page.

How many children are diagnosed with retinoblastoma?

Retinoblastoma makes up 2% of all cancers diagnosed in children before the age of 15. It is the most common eye cancer in children. An estimated 200 to 300 children in the United States will be diagnosed annually with the disease.

Most children who are diagnosed with retinoblastoma are younger than 6 years old. The average age of diagnosis is 2. Girls and boys are diagnosed at equal rates, as are children of different races and ethnicities. Generally, 3 out of 4 children with retinoblastoma have the disease in 1 eye. The disease is found equally in the left eye and right eye. About 25% of children with retinoblastoma have the disease in both eyes. About 70% to 80% of eyes can be saved in children with the disease in both eyes.

What is the survival rate for children with retinoblastoma?

There are different types of statistics that can help doctors evaluate a person’s chance of recovery from retinoblastoma. These are called survival statistics. A specific type of survival statistic is called the relative survival rate. It is often used to predict how having cancer may affect life expectancy. Relative survival rate looks at how likely people with retinoblastoma are to survive for a certain amount of time after their initial diagnosis or start of treatment compared to the expected survival of similar people without this cancer.

Example: Here is an example to help explain what a relative survival rate means. Please note this is only an example and not specific to this type of cancer. Let’s assume that the 5-year relative survival rate for a specific type of cancer is 90%. “Percent” means how many out of 100. Imagine there are 1,000 people without cancer, and based on their age and other characteristics, you expect 900 of the 1,000 to be alive in 5 years. Also imagine there are another 1,000 people similar in age and other characteristics as the first 1,000, but they all have the specific type of cancer that has a 5-year survival rate of 90%. This means it is expected that 810 of the people with the specific cancer (90% of 900) will be alive in 5 years.

It is important to remember that statistics on the survival rates for children with retinoblastoma are only an estimate. They cannot tell an individual person if cancer will or will not shorten their life. Instead, these statistics describe trends in groups of people previously diagnosed with the same disease, including specific stages of the disease.

The 5-year relative survival rate for children with retinoblastoma is 97%.

The survival rates for retinoblastoma vary based on several factors. These include the stage of cancer, a person’s age and general health, and how well the treatment plan works.

Experts measure relative survival rate statistics for children with retinoblastoma every 5 years. this means the estimate may not reflect the results of advancements in how children with retinoblastoma are diagnosed or treated from the last 5 years. Talk with your child’s doctor if you have any questions about this information. Learn more about understanding statistics .

Statistics adapted from the American Cancer Society and St. Jude Children’s Research Hospital websites. Additional source was : Seigel R, et al.: Cancer Statistics 2023 . CA: A Cancer Journal for Clinicians . 2023 Jan; 73(1):17–48. doi/full/10.3322/caac.21763 . (All sources accessed March 2023.)

The next section in this guide is Medical Illustrations . It offers drawings of the parts of the eye often affected by retinoblastoma. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find out more about the factors that increase the chance of developing retinoblastoma. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

Currently, the only known cause of retinoblastoma is a genetic mutation, or change in the genes. This genetic form of retinoblastoma accounts for about 40% of cases and always occurs in very young children, typically 1 year old or younger. When retinoblastoma affects both eyes, it is always a genetic condition. Despite the genetic link, only 10% to 15% of children with retinoblastoma have a family history of the disease. Rarely, the genetic form occurs in only 1 eye.

Children who have had bilateral retinoblastoma or the hereditary form of unilateral retinoblastoma are at an increased risk for developing other types of cancer. The risk of an additional tumor is higher for children who receive radiation therapy (see Types of Treatment ) to the orbit (eye socket) to preserve vision or to other parts of the body where the tumor has spread. If a newborn baby has a family history of retinoblastoma, the baby should be examined shortly after birth by an ophthalmologist who is experienced in treating cancers of the eye. An ophthalmologist is a medical doctor who specializes in eye care.

About 60% of children with retinoblastoma do not have the genetic form. They develop a single tumor in only 1 eye, and they have no increased risk of an additional tumor later in life.

The next section in this guide is Symptoms and Signs . It explains what changes or medical problems retinoblastoma can cause. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, cancer. They also do tests to learn if cancer has spread to another part of the body from where it started. If the cancer has spread, it is called metastasis. Doctors may also do tests to learn which treatments could work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. However, the diagnosis process is different for retinoblastoma. A diagnosis for retinoblastoma is never made by a biopsy when the tumor is confined to the eye or eyes. Instead, the diagnosis of retinoblastoma is made after a detailed examination of the affected eye or eyes by an experienced ophthalmologist.

How retinoblastoma is diagnosed

There are many tests used for diagnosing retinoblastoma. Not all tests described here will be used for every person. Your child's doctor may consider these factors when choosing a diagnostic test:

• The type of cancer suspected

• Your child's signs and symptoms

• Your child's age and general health

• The results of earlier medical tests

The next step after observing any symptoms (see Symptoms and Signs ) is to have the child examined by a specialist, who will do a thorough ophthalmic examination to check the retina for a tumor. Depending on the child’s age, either a local or general anesthetic is used during the eye examination. Anesthetic is a medication that blocks the awareness of pain.

The specialist will make a drawing or take a photograph of the tumor in the eye to provide a record for future examinations and treatment. Additional tests may also be done to locate or confirm the presence of a tumor.

In addition to a physical examination, the following tests may be used to diagnose retinoblastoma:

• Ultrasound. An ultrasound uses sound waves to create pictures of the internal organs. A transmitter that emits sound waves is moved over the child’s body. A tumor generates different echoes of the sound waves than normal tissue does, so when the waves are bounced back to a computer and changed into images, the doctor can locate a mass inside the body. The procedure is painless.

• Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size or help the doctor find cancer outside of the eye. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow. CT scans are used less commonly in diagnosing retinoblastoma because of the concern raised by the radiation associated with this type of scan.

• Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the brain and spinal column. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow. An MRI is increasingly recommended because it avoids the radiation associated with CT scans and better shows the structures of the eye, orbit, and brain.

• MRI or CT scan of the brain. These tests may be recommended to find out if there is an abnormality of the pineal gland, which is a small gland in the brain that regulates the body’s response to light. It is recommended that these scans be performed once every 6 months until age 5 for children with the genetic form of retinoblastoma, which includes those with bilateral disease and those with unilateral disease who have a family history of the disease. Very young children with a tumor in 1 eye who do not have a family history of the disease may also be at risk, and these tests may be recommended. Scans may also be recommended years after treatment for children who have received external-beam radiation therapy (see Types of Treatment ), either as a baseline in case of problems in the future or to determine the cause of a new symptom or sign of a problem.

If there are any additional signs found during the physical examination, the doctor may recommend more tests to determine if the cancer has spread elsewhere in the body.

• Blood tests. These tests evaluate the blood and check for problems with the liver and kidneys. The doctor may also look at the blood for changes in chromosome 13. Chromosomes are the parts of a cell that contain genes. In a few cases of retinoblastoma, these genes are either missing or nonfunctional. Molecular analysis of the gene is now possible in a few medical centers to determine changes that are not visible on an ordinary chromosome analysis.

• Lumbar puncture (spinal tap). A lumbar puncture is a procedure in which a needle is used to take a sample of cerebral spinal fluid (CSF) to look for cancer cells, blood, or tumor markers, which are substances found in higher than normal amounts in the blood, urine, or body tissues of people with certain kinds of cancer. CSF is the fluid that flows around the brain and the spinal cord. Doctors generally give an anesthetic to numb the child’s lower back before the procedure.

• Bone marrow aspiration and biopsy. These 2 procedures are similar and often done at the same time to examine the bone marrow. Bone marrow is the spongy, fatty tissue found inside larger bones that produces blood cells. Bone marrow has both a solid and a liquid part. A bone marrow aspiration removes a sample of the fluid with a needle. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle.

  A pathologist then analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. A common site for a bone marrow aspiration and biopsy is the pelvic bone, which is located in the lower back by the hip. Doctors generally give anesthesia beforehand to numb the area and to lessen the pain.

• Hearing test. Children with retinoblastoma receiving specific types of chemotherapy (see Types of Treatment ) may have their hearing tested to make sure the drugs are not causing hearing loss. This may also be called an audiology test.

Learn more about preparing your child for medical tests .

After diagnostic tests are done, your child's doctor will review the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer. This is called staging.

The next section in this guide is Stages. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will learn about the different types of treatments doctors use for children with retinoblastoma. Use the menu to see other pages.

In general, cancer in children is uncommon. This means it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That is why more than 60% of children with cancer are treated as part of a clinical trial. A clinical trial is a research study that tests a new approach to treatment. The “standard of care” is the best treatments known based on previous clinical trials. Clinical trials may test such approaches as a new drug, a new combination of existing treatments, or new doses of current therapies. The health and safety of all children participating in clinical trials are closely monitored.

To take advantage of these newer treatments, children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. An ocular oncologist is a doctor who specializes in treating eye cancer. If a pediatric cancer center is not nearby, general cancer centers sometimes have pediatric specialists who are able to be part of your child’s care.

How retinoblastoma is treated

In many cases, a team of doctors works with a child and their family to provide care. This is called a multidisciplinary team . Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, dietitians, physical and occupational therapists, social workers, and counselors. Special activities and programs to help the child and family cope may also be available.

Several types of therapies are used for retinoblastoma, and more than 90% of children can be cured. In addition to curing the retinoblastoma, an important goal of treatment is the preservation of vision. Many of the treatment approaches described below have become available as a result of clinical trials. The Children’s Oncology Group has recently developed clinical trials about treatment for retinoblastoma. (Please note this link takes you to another website.)

Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, the family’s preferences, and the patient’s overall health. Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Talk with your child’s doctor about the goals of each treatment and what your child can expect while receiving the treatment. These types of talks are called "shared decision-making." Shared decision-making is when your family and your child's doctors work together to choose treatments that fit the goals of your child's care. Shared decision-making is particularly important for retinoblastoma because there are different treatment options. Learn more about making treatment decisions .

The common types of treatments used for retinoblastoma are described below. Your child's care plan also includes treatment for symptoms and side effects, an important part of cancer care.

• Surgery

• Radiation therapy

• Cryotherapy

• Laser therapy

• Chemotherapy

• Bone marrow transplantation/stem cell transplantation

• Physical, emotional, and social effects of cancer

• Remission and the chance of recurrence

• If treatment does not work

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. Surgery to remove the eye is called enucleation. Your child's ocular oncologist will determine if surgery is the best option to treat your child's retinoblastoma.

Children with a tumor in 1 eye only can often be cured with this treatment. For children with a tumor in both eyes, enucleation is only used if other types of treatment to preserve vision are not possible. Surgical treatment for retinoblastoma should be performed by a specially trained pediatric ophthalmologist. Talk with your child's pediatric ophthalmologist or ocular oncologist about the recovery and possible side effects for the surgery your child is having. Learn more about the basics of cancer surgery .

If enucleation is the best treatment to cure the cancer, there will be some loss of eyesight. This may result in trouble with depth perception, but most children adapt well to these differences over time. Talk with your child's doctor about what to expect if enucleation is recommended and what support services are available to help your child adapt to any visual changes.

Many people worry about what their child will look like when they have an eye removed. Your child can have cosmetic surgery to fill the area left by the missing eye. They will be fitted with an artificial eye called a prosthesis. The prosthesis will look and behave almost the same as a natural eye. The artificial eye will move along with the person's remaining eye, though not as much as a natural eye moves. Most people will be unable to tell the difference. If enucleation is required, talk with your child's doctor about a prosthesis.

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Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. Proton beam radiation therapy is a type of external-beam radiation therapy that uses protons rather than x-rays. At high energy, protons can destroy cancer cells. Proton beam radiation therapy is a targeted way of delivering radiation therapy, which could decrease both the severity and frequency of short-term and long-term side effects. However, this treatment is not widely available in all areas.

Additionally, there is a type of internal radiation therapy, also called brachytherapy, for retinoblastoma called radioactive plaque therapy. Radioactive plaque therapy is the delivery of radiation therapy directly to the eye with a disc containing radiation.

Fatigue, drowsiness, nausea, vomiting, and headaches are common side effects of radiation therapy, and these usually go away after treatment. Radiation therapy in young children can interfere with the body’s normal development, including growth of the bones around the eye, depending on the dose the child received. The increased risk of additional tumors later in life for children with the hereditary form of retinoblastoma (see Risk Factors ) is further increased after external-beam radiation therapy. These effects are not seen after radioactive plaque therapy.

Talk with your child's health care team about the potential side effects of your child's radiation therapy plan. Learn more about the basics of radiation therapy .

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Cryotherapy

Cryosurgery, also called cryotherapy or cryoablation, uses liquid nitrogen to freeze and destroy cancer cells. More than 1 freezing may be needed.

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Laser therapy

Laser therapy uses heat in the form of a laser to shrink a smaller tumor. It may be called thermotherapy or "TTT" for transpupillary thermotherapy. It may be used alone or in addition to cryotherapy or radiation therapy. Photocoagulation is a different type of laser therapy that uses light to shrink tumors.

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Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells. It may be used to shrink an eye tumor. Chemotherapy is given by a pediatric oncologist or a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time.

Chemotherapy often makes it possible to completely get rid of any remaining smaller tumors with the following focal (localized) measures, described above:

• Thermotherapy or photocoagulation (laser therapy)

• Cryotherapy

• Radioactive plaque therapy

Chemoreduction uses chemotherapy to reduce the size of the tumor. This is a treatment approach that is often used for children with retinoblastoma in both eyes in the hope of avoiding enucleation and preserving vision in at least 1 eye. The ophthalmologist, working closely with the pediatric oncologist, will determine if this treatment is appropriate.

Children with low-risk disease and some children with intermediate-risk disease may be able to avoid chemotherapy after surgery or reduce the intensity of chemotherapy. Talk with your child's doctor about whether this approach is recommended for your child. Both doctors will regularly monitor how well the treatment is working and may recommend additional treatment to prevent the cancer from returning.

The drugs used most often for retinoblastoma are vincristine (Oncovin, Vincasar PFS), carboplatin (Paraplatin), and etoposide (Toposar, VePesid). Depending on the extent of the tumor, a combination of 2 or more drugs may be recommended. All chemotherapy has side effects that occur during treatment. Some drugs may also cause specific long-term side effects. Talk with your child's doctor about the specific drugs used and the possible short-term and long-term side effects before treatment begins.

There are different ways to give chemotherapy for retinoblastoma.

• Systemic chemotherapy is when medication is given through the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

• Intra-arterial chemotherapy (IA therapy) is chemotherapy that is delivered directly into the artery or the blood vessel that supplies the affected eye. IA therapy is done by inserting a catheter in the child’s groin area. The doctor moves the catheter with the help of imaging technology to reach the blood vessel in the head that directly supplies the eye. This procedure may be used in an effort to save the eye in children with advanced disease if the tumor is still confined to the eye. This method of giving chemotherapy with 1 or several drugs is being used both in children with 1 affected eye or 2 affected eyes. This method is performed by an interventional radiologist.

• Intravitreal chemotherapy (IVIT therapy) is chemotherapy that is delivered by directly injecting it into the cavity of the eye. IVIT therapy is effective in treating vitreous seeds, which are found in the fluid inside the eye. Vitreous seeds are difficult to treat with systemic chemotherapy alone.

Learn more about the basics of chemotherapy .

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Bone marrow transplantation/stem cell transplantation

For some children with stage IV extraocular retinoblastoma , a bone marrow transplant may be recommended. The child is first treated with high doses of chemotherapy (and/or radiation therapy) to destroy as many cancer cells as possible, then the transplantation takes place.

A bone marrow transplant is a medical procedure in which bone marrow that contains cancer is replaced by highly specialized cells. These cells, called hematopoietic stem cells, develop into healthy bone marrow. Hematopoietic stem cells are blood-forming cells found both in the bloodstream and in the bone marrow. This procedure is also called a stem cell transplant or hematopoietic stem cell transplant.

Before recommending transplantation, doctors will talk with the patient about the risks of this treatment. They will also consider several other factors, such as results of any previous treatment and the patient’s age and general health.

There are 2 types of hematopoietic stem cell transplantation depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO). ALLO uses donated stem cells, while AUTO uses the patient’s own stem cells. In both types, the goal is to destroy all of the cancer cells in the marrow, blood, and other parts of the body using high doses of chemotherapy and/or radiation therapy, and then allow replacement blood stem cells to create healthy bone marrow.

Side effects depend on the type of transplant, your child’s general health, and other factors. Learn more about the basics of stem cell and bone marrow transplantation .

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Physical, emotional, and social effects of cancer

Cancer and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your child's care that is included along with treatments intended to slow, stop, or eliminate the cancer.

Palliative care focuses on improving how your child feels during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative care along with treatment for the cancer often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. Your child may also receive palliative treatments similar to those meant to get rid of the cancer, such as chemotherapy, surgery, or radiation therapy.

Before treatment begins, talk with your child's doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

During treatment, your child's health care team may ask you to answer questions about your child's symptoms and side effects and to describe each problem. Be sure to tell the health care team if your child experiences a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.

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Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. Although many remissions are permanent, it is important to talk with your child’s doctor about the possibility of the cancer returning. Understanding your child’s risk of recurrence and the treatment options may help you and your child feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence .

If the cancer returns after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

If a recurrence happens, a new cycle of testing will begin again to learn as much as possible about it. After this testing is done, you and your child’s doctor will talk about the treatment options. If retinoblastoma returns after treatment, the treatment plan depends on where the cancer recurred and how aggressive the new tumor is. The doctor may recommend surgery, radiation therapy, chemotherapy, and/or focal measures, such as photocoagulation, thermotherapy, or cryotherapy (see above). The doctor may suggest clinical trials that are studying new ways to treat recurrent retinoblastoma. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent cancer and their families sometimes experience emotions such as disbelief or fear. Parents are encouraged to talk with their child's health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence .

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If treatment does not work

Although treatment is successful for most children with retinoblastoma, sometimes it is not. If a child’s cancer cannot be cured or controlled, this is called advanced or terminal cancer. This diagnosis is stressful, and advanced cancer may be difficult to discuss. However, it is important to have open and honest conversations with your child’s health care team to express your family’s feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help.

Hospice care is designed to provide the best possible quality of life for people who are expected to live less than 6 months. Parents or guardians are encouraged to talk with the health care team about hospice options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Some children may be happier and more comfortable if they can attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced cancer care planning .

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child .

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The next section in this guide is About Clinical Trials . It offers more information about research studies that are focused on finding better ways to care for children with cancer. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will read about the scientific research being done to learn more about retinoblastoma and how to treat it. Use the menu to see other pages.

Doctors are working to learn more about retinoblastoma, ways to prevent it, how to best treat it, and how to provide the best care to children diagnosed with retinoblastoma. The website of the Children’s Oncology Group offers a list of clinical trials researching the treatment options listed below, and these clinical trials may be open for some children with retinoblastoma. Always talk with your child’s doctor about the best diagnostic and treatment options for your child.

• More intensive treatment with chemotherapy and radiation therapy to preserve vision for children with advanced disease in both eyes

• Improved ways to deliver chemotherapy directly to the eye so that children can be spared the side effects of receiving chemotherapy to the entire body

• Less intensive treatment with chemotherapy for small tumors

• Better ways of determining when to use chemotherapy after enucleation, and whether chemotherapy can prevent the spread of a tumor

• Aggressive treatment to cure children who have had a recurrence of retinoblastoma or have had the cancer spread outside the eye

• Better ways of reducing symptoms and side effects of current retinoblastoma treatments in order to improve patients’ comfort and quality of life

Looking for More About the Latest Research?

If you would like more information about the latest areas of research in retinoblastoma, explore these related items that take you outside of this guide:

• To find clinical trials specific to your child's diagnosis, talk with your child’s doctor or search online clinical trial databases .

• Visit the website of Conquer Cancer, the ASCO Foundation , to find out how to help support cancer research. Please note that this link takes you to a different ASCO website.

The next section in this guide is Coping with Treatment . It offers some guidance on how to cope with the physical, emotional, social, and financial changes that cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will read about your child’s medical care after cancer treatment is completed and why this follow-up care is important. Use the menu to see other pages.

Care for children diagnosed with cancer does not end when active treatment has finished. Your child’s health care team will continue to check that the cancer has not come back, manage any side effects, and monitor your child’s overall health. This is called follow-up care. All children treated for cancer, including retinoblastoma, should have life-long, follow-up care.

Your child’s follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your child’s recovery in the months and years ahead. Once a child has been free of retinoblastoma for 2 to 4 years after treatment and is considered cured, the emphasis during periodic follow-up visits changes. Pediatric oncologists will focus on enhancing the quality of the child’s life, including addressing developmental and emotional concerns.

If enucleation took place, most young children adapt well to the loss of 1 eye. Rarely, both eyes will need to be removed to save the child’s life. If both eyes are removed, the local educational system is required to provide special services. Parents are encouraged to investigate the school’s services and advocate on their child’s behalf. Talking with an oncology social worker may help. Other rehabilitation services may also be needed to help the child cope with the vision loss.

Cancer rehabilitation after retinoblastoma treatment could mean any of a wide range of services, such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent as possible. Learn more about cancer rehabilitation.

Learn more about the importance of follow-up care .

Watching for recurrence

One goal of follow-up care is to check for a recurrence, which means that the cancer has come back. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms.

During follow-up care, a doctor familiar with your child’s medical history can give you personalized information about the risk of recurrence. Your child's doctor will ask specific questions about your child’s health. Some children may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the type and stage of cancer first diagnosed and the types of treatment given.

The anticipation before having a follow-up test or waiting for test results may add stress to you or a family member. This is sometimes called “scanxiety.” Learn more about how to cope with this type of stress .

Managing long-term and late side effects of childhood cancer

Sometimes, side effects may linger beyond the active treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years after treatment has ended. Late effects can occur almost anywhere in the body. They include physical problems, such as heart and lung problems, and second cancers, which is a new cancer that happens in someone who has had cancer before. They also include emotional problems, such as anxiety and depression, and problems with memory, thinking, attention, and learning.

Based on the type of treatment your child received and whether the child has the genetic form of retinoblastoma, the doctor will determine what evaluations are needed to check for long-term effects. This may include imaging studies (computed tomography scans or magnetic resonance imaging) and blood tests. Counseling will also be provided for children who have an increased risk of additional tumors later in life, such as those with retinoblastoma in both eyes and those with retinoblastoma in 1 eye who have a family history of the disease. Yearly visits to specialized ophthalmologic and medical oncologists are necessary in order to fully monitor the child’s recovery and to find any secondary cancer in its earliest stages. Follow-up care should also address any developmental or emotional concerns from having had cancer and its treatment.

The Children's Oncology Group (COG) has studied the physical and psychological effects that childhood cancer survivors face. Based on these studies, COG has created recommendations for long-term follow-up care for childhood, adolescent, and young adult cancer survivors that can be found on a separate website of COG: www.survivorshipguidelines.org .

Keeping a child’s personal health record

You are encouraged to organize and keep a personal record of your child’s medical information. The doctor will help you create this. That way, as the child enters adulthood, they have a clear, written history of the diagnosis, the treatments, and the doctor’s recommendations about the schedule for follow-up care. The American Society of Clinical Oncology (ASCO) offers forms to help keep track of the cancer treatment your child received and develop a survivorship care plan when treatment is completed.

Some children continue to see their oncologist, while others transition back to the care of their pediatrician, primary care doctor, or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your family’s personal preferences. Talk with the health care team about your child’s ongoing medical care and any concerns you have about their future health.

If a doctor who was not directly involved in your child’s cancer care will lead the follow-up care, be sure to share the cancer treatment summary and survivorship care plan forms with them and with all future health care providers. Details about the specific cancer treatment given are very valuable to the health care professionals who will care for your child throughout their lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find some questions to ask the health care team, to help you better understand your child’s diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with the health care team is important to make informed decisions about your child’s health care. These suggested questions are a starting point to help you learn more about your child’s cancer care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your child's next appointment, or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your child’s care. It may also be helpful to ask a family member or friend to come with you to appointments to help take notes.

Questions to ask after getting a diagnosis

• Does my child have the genetic form of retinoblastoma?

• What is the stage of the disease? What does this mean?

• Can you explain my child’s pathology report (laboratory test results) to me?

Questions to ask about choosing a treatment and managing side effects

• What are the treatment options?

• What clinical trials are available for my child? Where are they located, and how do I find out more about them?

• Who will be part of my child’s health care team, and what does each member do?

• Who will be leading my child’s overall treatment?

• What treatment plan do you recommend? Why?

• What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?

• What is the likelihood that the recommended treatment can save my child’s vision?

• If enucleation is needed, what support services are available to help my child adjust to the partial/total loss of vision?

• If enucleation is needed, how soon can my child receive a prosthesis (artificial eye)?

• If recommended, what can I expect chemotherapy to accomplish in the treatment of my child’s cancer? How will chemotherapy be given?

• If recommended, what are the relative benefits and risks of radiation therapy in treating my child?

• What are the common side effects of each treatment, both in the short term and long term?

• What can be done to prevent or relieve the side effects?

• How will this treatment affect my child’s daily life? Will they be able to go to school and perform their usual activities?

• Could this treatment affect my child’s ability to become pregnant or have children in the future? If so, should our family talk with a fertility specialist before cancer treatment begins?

• If I’m worried about managing the costs of cancer care, who can help?

• If I have questions or problems, who should I call?

Questions to ask about planning follow-up care

• What is the chance that the cancer will come back? Should I watch my child for specific signs or symptoms?

• What long-term side effects or late effects are possible based on the cancer treatment my child received?

• What follow-up tests will my child need, and how often will those tests be needed?

• How do I get a treatment summary and survivorship care plan to keep in my child's personal records?

• If I have questions or problems, who should I call?

• Who will be leading my child's follow-up care?

• What survivorship support services are available to my child? To my family?

The next section in this guide is Additional Resources. It offers more resources on this website that may be helpful to you. Use the menu to choose a different section to read in this guide.