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Retinoblastoma - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Childhood Retinoblastoma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

Retinoblastoma is a rare cancer that begins in the part of the eye called the retina. The retina is a thin layer of nerve tissue that coats the back of the eye and enables the eye to see. Most often, retinoblastoma is unilateral, meaning it occurs only in one eye. However, it may be present in both eyes, called bilateral. If retinoblastoma spreads, it can spread to the lymph nodes, bones, or the bone marrow (the soft, spongy-like material found inside large bones). Rarely, it involves the central nervous system (CNS; brain and spinal cord).

Children may be born with retinoblastoma, but the disease is rarely diagnosed at birth. Most children who begin treatment before the retinoblastoma has spread beyond the eye are cured. An important goal of treatment in children with retinoblastoma is preserving vision.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items. Please note these links take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a one-page fact sheet (available in PDF) that offers an easy-to-print introduction to this type of cancer.

To continue reading this guide, use the menu on the side of your screen to select another section.

Retinoblastoma - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find information about how many children learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

This year, an estimated 280 children in the United States will be diagnosed with retinoblastoma. Most children who are diagnosed with retinoblastoma are younger than five years old. Retinoblastoma makes up 3% of all cancers diagnosed in children before the age of 15.

The five-year survival rate is the percentage of children who survive at least five years after the cancer is detected, excluding those who die from other diseases. The five-year survival rate for children with retinoblastoma is 99%, but it depends on several factors, including whether the cancer has spread to other parts of the body.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of children of this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with retinoblastoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's (ACS) publication, Cancer Facts and Figures 2014.

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Retinoblastoma - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find out more about what factors increase the chance of this type of cancer. To see other pages, use the menu on the side of your screen.

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 (change). This genetic form of retinoblastoma accounts for about 40% of cases and always occurs in very young children (rarely older than one year). 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 one eye.

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

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

To continue reading this guide, use the menu on the side of your screen to select another section.

Retinoblastoma - Childhood - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

Children with retinoblastoma often experience the following symptoms or signs. Sometimes, children with retinoblastoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your child’s doctor. Sometimes, a doctor finds retinoblastoma during a well-baby examination. Most often, however, parents notice symptoms or signs such as:

  • A pupil that looks white or red, instead of the normal black
  • A crossed eye (looking either toward the ear or toward the nose)
  • Poor vision
  • A red, painful-looking eye
  • An enlarged pupil
  • Different-colored irises

Your child’s doctor will ask questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long your child has been experiencing the symptom(s) and how often.

If cancer is diagnosed, relieving symptoms and side effects remains an important part of your child’s cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Retinoblastoma - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. Although a biopsy (the removal of a small amount of tissue for examination under a microscope) is the way to make a definitive diagnosis for most types of cancer, this is usually not possible for retinoblastoma, and the doctor will suggest other ways to make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of cancer suspected
  • Signs and symptoms
  • Previous test results

The next step after observing any symptoms (see Symptoms) 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 (medication that blocks the awareness of pain) is used during the eye examination.

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 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 creates a three-dimensional picture of the inside of the child’s body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size or help the doctor find cancer outside of the eye. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein or given orally (by mouth) to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the brain and spinal column. MRIs may create more detailed pictures than CT scans and provide the specialist with a picture of the inside of the eye and the brain. A contrast medium (a special dye) may be injected into a patient’s vein or given orally to create a clearer picture.

MRI or CT scan of the brain. These tests may be recommended to find out if there is an abnormality of the pineal gland (a small gland in the brain that regulates the body’s response to light). It is recommended that these scans be performed once every six months until age five for children with the genetic form of retinoblastoma (those with bilateral disease and those with unilateral disease with a family history of the disease). Very young children with a tumor in one 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 Treatment), either as a baseline in case of problems in the future, or to determine the cause of a symptom or sign.

If there are any additional signs found during the physical examination, the doctor may recommend additional 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, and 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 ordinary chromosome analysis.

Lumbar puncture (spinal tap). A lumbar puncture is a procedure in which a doctor takes a sample of cerebral spinal fluid (CSF) to look for cancer cells, blood, or tumor markers (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 biopsy and aspiration. These procedures are performed to determine if any retinoblastoma cells have spread to the bone marrow and are often done at the same time. Bone marrow has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. A common site for a bone marrow biopsy and aspiration is the pelvic bone, which is located in the lower back by the hip. The skin in that area is usually numbed with medication beforehand, and other types of anesthesia may be used.

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

After these diagnostic tests are done, your doctor will review all of 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 helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.  

Retinoblastoma - Childhood - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

After a retinoblastoma has been found, the doctor will determine the extent of disease in the eye and if the disease has spread outside the eye. This is called staging, and it helps doctors plan treatment. First, the doctor will determine if the retinoblastoma is intraocular or extraocular.

Intraocular. This means that cancer occurs in one or both eyes, but has not spread into surrounding tissues or other parts of the body.

Extraocular. The cancer has spread to tissues around the eye or to other parts of the body.

Several intraocular staging systems have been in use for many years to help doctors plan treatment. However, the recent success of clinical trials (research studies) demonstrating the effectiveness of chemotherapy to shrink tumors has led to the development of a newer staging system called the International Classification System.

International (ABC) Classification System

Group A:

  • Small tumor(s) located only in the retina
  • No tumor is larger than 3 millimeters (mm)
  • No tumor is closer than 2 disc diameters (DD) from the fovea (the central “pit” of the retina) or 1 DD from the optic nerve
  • No vitreous seeding (tumor floating in the eye) or retinal detachment

Group B:

  • Tumor(s) located only in the retina
  • Any location in the retina
  • No vitreous seeding
  • No retinal detachment more than 5 mm from tumor base

Group C:

  • Fine diffuse (spread throughout) or localized (located in one spot) vitreous seeding
  • Retinal detachment: more than Group B and up to total retinal detachment
  • No vitreous/sub-retinal “snowballs” or masses

Group D:

  • Massive vitreous/sub-retinal seeding
  • Vitreous or sub-retinal snowballs/masses
  • Retinal detachment: more than Group B and up to total retinal detachment

Group E:

No visual potential, or:

Presence of one or more:

  • Tumor in CB/anterior segment
  • Neovascular glaucoma (This usually results in increased pressure within the eye as a result of blood vessels growing into the anterior segment of the eye and represents an advanced stage of retinoblastoma.)
  • Vitreous hemorrhage (bleeding from the eye)
  • Phthisical/prephthisical (deteriorating) eye
  • Hyphema (blood in the anterior of the eye)/corneal staining
  • Orbital cellulitis-like presentation (the eye looks similar to the signs of an infection, but there is no infection)
  • Tumor anterior to anterior hyaloids (the tumor is behind the thin membrane that separates the vitreous (white gel-like part of the eye) from other parts of the eye)

Extraocular staging

This staging system applies to cancer that has spread outside of the eye, either to the tissues around the eye or the central nervous system (CNS), bone marrow, or lymph nodes.

Stage 0 (zero). This means that the cancer is in the eye (called intraocular) and has not spread outside of the eye. Patients with stage 0 disease should be classified based on the International Classification System, outlined above. Doctors recommend conservative treatment for this stage with no additional adjuvant therapy (meaning no additional treatment after surgery; see the Treatment section for additional information).

Stage I. A classification of stage I means that eye has already been removed and there is some microscopic spread to the optic nerve. These patients need adjuvant chemotherapy after surgery.

Stage II. This stage means that the tumor has spread to the optic nerve or the sclera (the white part of the eye). Patients with this stage need adjuvant chemotherapy and possibly radiation therapy (see the Treatment section for more information on these treatments.)

Stage III. This means that the cancer has spread to the lymph nodes or the bony cavity that surrounds the eyeball. It is divided into two substages called stage IIIa and stage IIIb, depending on where the tumor has spread. The treatment for this stage involves both chemotherapy and radiation therapy.

Stage IV. In stage IV, the tumor has spread to distant areas of the body outside the eye, via the lymphatic system and blood vessels. Stage IV is divided into substages of IVa and IVb. Stage IVb is further divided into IVb1, IVb2, and IVb3, depending on the location of the spread. This stage of extraocular retinoblastoma is treated with high-dose chemotherapy followed by stem cell rescue. This means that the child’s stem cell/bone marrow cells are replaced with healthy cells. Learn more about bone marrow/stem cell transplantation.  Radiation therapy may also be used in addition to high-dose chemotherapy for some patients with stage IV disease. 

Recurrent. Recurrent cancer is cancer that has come back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

Information about the cancer’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Retinoblastoma - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will learn about the different ways doctors use to treat people with this type of cancer. To see other pages, use the menu on the side of your screen.

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best proven treatments available) with newer treatments that may be more effective. Studying new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, all 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. In many cases, a team of doctors works with a child and the 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, nutritionists, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your 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 these treatment approaches described below have become available as a result of clinical trials. The Children’s Oncology Group has recently developed treatment clinical trials that include retinoblastoma.

Descriptions of the most common treatment options for retinoblastoma are listed below. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Take time to learn about your child’s treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with the doctor and what your child can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation. Surgery to remove the eye is called enucleation. Children with a tumor in one eye only can often be cured with this treatment. For children with a tumor in both eyes, enucleation is used only if the ocular oncologist (a doctor who specializes in eye cancer) determines that preserving vision using other treatment is not possible. Surgical treatment for retinoblastoma should be performed by a specially trained pediatric ophthalmologist.

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

Many people worry about what they will look like when they have an eye removed. The cosmetic surgery available today usually has good cosmetic results. To fill the area left by the missing eye, the person is fitted for an artificial eye, called a prosthesis. The prosthesis will look and behave almost the same as a natural eye. For example, the artificial eye will move along with the person’s remaining eye, just not as much as a natural eye moves. Family members may be able to tell that the eye is not real, but it is unlikely that strangers will know. If enucleation is required, talk with your doctor about a prosthesis; it may take many weeks for patients to receive the prosthesis. Learn more about cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. Radioactive plaque therapy, also called internal radiation therapy or brachytherapy, is the delivery of radiation therapy directly to the eye with a disc containing radiation. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Fatigue, drowsiness, nausea, vomiting, and headache are common side effects of radiation therapy, and these usually go away after treatment. Radiation therapy in young children can interfere with normal growth, including growth of the bones around the eye, depending on the dose. The increased risk of additional tumors later in life for children with the hereditary form of retinoblastoma is further increased after external-beam radiation therapy. These effects are not seen after radioactive plaque therapy. Learn more about radiation therapy.

Cryotherapy

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

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), and 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.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide and may be used to shrink an eye tumor. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a pediatric oncologist or a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen usually consists of a specific number of cycles given over a set period of time.

Chemotherapy is also delivered through the blood vessel feeding the eye and the tumor by inserting a catheter in the child’s groin area. The doctor maneuvers the catheter with the help of imaging technology to reach the blood vessel in the head. This procedure is used in a few medical centers for children with advanced retinoblastoma with some success in an attempt to prevent removal of the affected eye.

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

  • Thermotherapy or photocoagulation (laser therapy)
  • Cryotherapy
  • Radioactive plaque therapy

Chemoreduction (using chemotherapy to reduce the size of the tumor) 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 one 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 postoperative chemotherapy or reduce the intensity of chemotherapy. Talk with the doctor about whether this approach is appropriate for your child. Both doctors will regularly monitor how the treatment is affecting the cancer 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 two or more drugs will be recommended. All chemotherapy has side effects that occur during treatment. Some drugs have the potential for specific long-term side effects. Talk with your doctor about the specific drugs used and the possible long-term side effects before treatment begins.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions by using searchable drug databases.

Stem cell rescue/transplantation

For some children with Stage IV extraocular retinoblastoma, a stem cell 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 stem cell transplant is a medical procedure in which healthy, highly specialized cells, called hematopoietic stem cells, are given to a patient, either to replace bone marrow that contains cancer or to help the patient’s bone marrow recover after other cancer treatment. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the stem cells in the blood that are typically being transplanted, not the actual bone marrow tissue.

Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as results of any previous treatment and the patient’s age and general health. Learn more about bone marrow and stem cell transplantation.

Getting care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with your child’s doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of your child’s specific treatment plan and supportive care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child is experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care.   

Recurrent retinoblastoma

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

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. Although many remissions are permanent, it’s important to talk with your child’s doctor about the possibility of the cancer returning. Understanding the 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).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the cancer’s stage has changed. After testing is done, you and your child’s doctor will talk about your 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 also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

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

If treatment fails

Although treatment is successful for the majority of children with cancer, 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 it may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to 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.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Retinoblastoma - Childhood - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat children with retinoblastoma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and managing the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating retinoblastoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with retinoblastoma.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for retinoblastoma, learn more in the Latest Research section.

Children who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that parents with a child participating in a clinical trial talk with their child’s doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the child leaves the clinical trial before it ends.

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.

Retinoblastoma - Childhood - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. To see other pages, use the menu on the side of your screen.

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 this disease. The Children’s Oncology Group has begun clinical trials to research 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 diagnostic and treatment options best for your child.

  • More intensive treatment with chemotherapy and radiation treatment 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 which children who have had enucleation need chemotherapy, and whether chemotherapy can prevent the spread of a tumor
  • Aggressive treatment to cure children who have had a recurrence of retinoblastoma or when cancer has 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 additional information about the latest areas of research regarding retinoblastoma, explore these related items that take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.
  • Review a consumer-friendly summary of research published in the Journal of Clinical Oncology, ASCO’s peer-reviewed journal.
  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for all childhood cancers (called pediatric cancers on this website), including retinoblastoma. Please note this link takes you to a separate ASCO website. 

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.

Retinoblastoma - Childhood - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for retinoblastoma are described in detail within the Treatment section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your child’s overall health.

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends often play an important role in the care of a child with retinoblastoma. Learn more about caregiving.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your child’s doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.

Retinoblastoma - Childhood - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will read about your child’s medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for retinoblastoma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for cancer, including retinoblastoma, should have life-long, follow-up care.

At first, follow-up visits will focus on making sure the cancer is gone and hasn’t returned. Once a child has been free of retinoblastoma for two to four years after treatment, and is considered cured, the emphasis during periodic follow-up visits changes. Pediatric oncologists will focus on the quality of the child’s life, including developmental and emotional concerns.

If enucleation took place, most young children adapt well to the loss of one 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. Other rehabilitation services may also be needed to help the child cope with the vision loss.

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 (CT scan or MRI) 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 one 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. Learn more about childhood cancer survivorship.

The child’s family is encouraged to organize and keep a record of the child’s medical information. That way, as the child enters adulthood, he or she has a clear, written history of the diagnosis, the treatment given, and the doctor’s recommendations about the schedule for follow-up care. The doctor’s office can help you create this. This information will be valuable to doctors who care for your child during his or her lifetime. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment your child received and develop a survivorship care plan once treatment is completed.

Children who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs. Learn more about the next steps to take in survivorship.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.  

Retinoblastoma - Childhood - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find some questions to ask your child’s doctor or other members of the health care team, to help you better understand your child’s diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor 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 the next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your child’s care.

  • 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?
  • What are the treatment options?
  • What clinical trials are open to my child?
  • Who will be part of my child’s health care team, and what does each member do?
  • Who will be coordinating my child’s overall treatment and follow-up care?
  • What treatment plan do you recommend? Why?
  • What is the goal 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?
  • 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?
  • How will this treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her usual activities?
  • Could this treatment affect my child’s ability to become pregnant or have children? If so, should our family talk with a fertility specialist before cancer treatment begins?
  • If I’m worried about managing the costs related to my child’s cancer care, who can help me with these concerns?
  • What follow-up tests will my child need, and how often are they needed?
  • What support services are available to my child? To my family?
  • Whom should I call with questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.  

Retinoblastoma - Childhood - Additional Information Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2013

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Childhood Retinoblastoma. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

- Search for a cancer specialist in your local area using this free database of doctors from the American Society of Clinical Oncology.

- Review dictionary articles to help understand medical phrases and terms used in cancer care and treatment.

- Read more about the first steps to take when newly diagnosed with cancer.

- Learn more about coping with the emotions that cancer can bring, including those within a family or a relationship.

- Find a national, not-for-profit advocacy organization that may offer additional information, services, and support for people with this type of cancer.

- Explore next steps a person can take after active treatment is complete.

This is the end of the Cancer.Net’s Guide to Childhood Retinoblastoma. Use the menu on the side of your screen to select another section to continue reading this guide.