What Is Retinoblastoma? A Plain-Language Guide to the Eye Cancer That Mostly Affects Young Children
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer
Retinoblastoma is a rare eye cancer that starts in the retina, the light-sensitive lining inside the eye [1]. It mostly affects very young children, usually diagnosed before age 2, most often in one eye [1]. A common sign is a white color in the center of the eye under light [1]. Treatment includes chemotherapy and laser or cold therapy [2].
What the research says
Most eyes can be saved with chemotherapy when the tumor is caught while still small. A 2026 review of retinoblastoma management reports that intravenous chemotherapy provides globe salvage with tumor control in 96% of Group A, 90% of Group B and Group C, 68% of Group D, and 32% of Group E eyes (groups run from smaller to larger, more advanced tumors). Plaque radiotherapy still has a role for tumors that do not respond to chemotherapy, and enucleation is kept for massive or high-risk disease. (Shields et al., Eye, 2026) [3]
Late recognition is a real problem in some countries. In a hospital-based study of 217 children with newly diagnosed retinoblastoma in Ethiopia, leukocoria (the white pupil glow) was the most common presenting sign (37.3%), tumors had already grown outside the eye in 40.1%, and the authors concluded that most children presented late. They called for campaigns to raise public awareness of the warning signs. (Sherief et al., Ocul Oncol Pathol, 2022) [4]
The white glow is the sign to act on. In a Spanish study of 38 children with retinoblastoma, leukocoria was the main sign (90% of cases), the average delay before diagnosis was 3.2 months, and only one death occurred among 25 patients followed for at least 5 years. The authors stressed close coordination between pediatricians and ophthalmologists. (Gargallo et al., Arch Soc Esp Oftalmol, 2018) [5]
Survival is high when caught early. In 483 children treated across 40 European countries, 2.5% died of retinoblastoma, but 75% of those whose tumor had spread outside the eye died; 58% overall lost an eye. (Bowman et al., Eye (Lond), 2026) [6]
Late diagnosis drives poor outcomes in lower-resource settings. In 958 children from 41 African countries, 3-year survival was 66.2% and 72.6% lost at least one eye, with the most advanced stage carrying over 6 times the risk of death. (Nishath et al., Br J Ophthalmol, 2025) [7]
What Is Retinoblastoma?
Retinoblastoma is a kind of eye cancer that starts as a growth of cells in the retina [1]. The retina is the light-sensitive lining on the inside of the eye [1]. It is made up of nerve tissue that senses light as it comes in through the front of the eye [1]. The retina then sends signals to the brain, and the brain interprets those signals as images [1].
To understand where this happens, it helps to know that the eye is a compact structure measuring about 1 inch (2.5 centimeters) in diameter [1]. The retina lines the inside of that structure, like film in a camera.
Retinoblastoma happens most often in young children. It is usually diagnosed before age 2 [1], and it most often affects one eye. Sometimes it happens in both eyes [1].
It is the most common eye cancer in children, although it remains rare overall [7].
There are several treatments for retinoblastoma. For most children, treatment does not require removing the eye to get rid of the cancer, and the outlook for children diagnosed with retinoblastoma is quite good [1].
What Does Retinoblastoma Look Like?
Retinoblastoma signs and symptoms include:
When Should You See a Doctor?
Make an appointment with a doctor or other healthcare professional if you notice any changes to your child's eyes that concern you [1]. The earlier the cancer is found, the more treatment options there are, and the better the chance of keeping the eye and preserving vision.
What Causes Retinoblastoma?
Retinoblastoma is caused by changes inside the cells in the eye [1]. It is not always clear what causes the changes that lead to this eye cancer.
Here is how it works in plain language. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate, and it also tells the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly, and the cancer cells keep living when healthy cells would die. This causes too many cells.
In retinoblastoma, this growth of cells happens in the retina. As the cancer cells build up, they can form a mass called a tumor [1]. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. When cancer spreads, it is called metastatic cancer. Retinoblastoma rarely spreads, especially if it is found early [1].
For most instances of retinoblastoma, it is not clear what causes the DNA changes that lead to cancer [1]. However, it is possible for children to inherit DNA changes from their parents [1], and these changes can increase the risk of retinoblastoma.
Who Is at Risk for Retinoblastoma?
Risk factor | What it means |
Young age | Retinoblastoma is most common in very young children [1] and is typically diagnosed by age 2. Retinoblastoma that happens later in life is very rare. |
Family DNA changes | DNA variations that increase the risk can be passed from parents to children. Children with these inherited changes tend to get retinoblastoma at a younger age and often in both eyes [1]. |
What Complications Can Happen After Treatment?
Two complications matter most for families of children with retinoblastoma.
Cancer that comes back. After treatment, there is a risk that the cancer might come back in the eye or near it [1]. For this reason, the healthcare team creates a follow-up plan that depends on the treatments your child received. A typical plan might involve eye exams every few months for the first few years after treatment [1].
Increased risk of other cancers. Children with the form of retinoblastoma that can run in families may have a higher risk of getting other kinds of cancer [1]. The cancers with increased risk include bone cancer, bladder cancer, breast cancer, Hodgkin lymphoma, lung cancer, melanoma, pineoblastoma, and soft tissue sarcoma [1]. The healthcare team might recommend tests to screen for these other types of cancer.
Can Retinoblastoma Be Prevented?
Retinoblastoma cannot be prevented [1], but families with a history of it can consider genetic testing and early eye exams.
Some retinoblastomas are caused by DNA changes that run in families. If retinoblastoma runs in your family, tell your healthcare professional. Together you might consider genetic testing to look for variations in your DNA that increase the risk of retinoblastoma [1]. Your health professional might refer you to a genetic counselor [1], a professional trained in genetics who can help you decide whether to undergo genetic testing.
If your children have an increased risk of retinoblastoma, care can be planned to manage that risk. For instance, eye exams may begin soon after birth [1]. That way, retinoblastoma may be diagnosed very early, when the cancer is small and has a greater chance of being cured.
If you have not had children but are planning to, talk with your healthcare team about your family history of retinoblastoma [1]. Genetic testing might help you and your partner understand whether there is a risk of passing DNA variations to your future children, and your healthcare team may have options to help manage this risk.
How Is Retinoblastoma Diagnosed?
Retinoblastoma diagnosis often starts with an eye exam [2]. Imaging tests can then help show the size of the cancer.
Eye exam. A healthcare professional carefully examines your child's eyes. This might involve testing your child's vision and using a special light to look inside the eye. Sometimes very young children find it hard to hold still for a thorough eye exam, so the healthcare team might recommend medicine to put the child in a sleep-like state so the exam can be completed [2].
Imaging tests. Imaging tests make pictures of the inside of the body. For retinoblastoma, they are used to look at the eye and the area around it. The images might show the size of the cancer and whether it has grown beyond the eye [2]. Imaging tests may include ultrasound and MRI, among others [2].
Genetic testing. Genetic testing uses a sample of blood or saliva to look for variations in the DNA [2]. For retinoblastoma, it looks for variations in a part of the DNA called the RB1 gene.
Everyone with retinoblastoma has variations in the RB1 gene in their cancer cells [2]. But some children have the RB1 gene variations in all of the cells in their bodies. This can happen if parents pass DNA variations to their child, or if something changes the RB1 gene as the child develops in the womb. If genetic testing shows RB1 variations in all of the cells in the body, this helps the healthcare team plan treatment. It also raises the risk of other kinds of cancer, and screening tests can help watch for them.
How Is Retinoblastoma Treated?
Common retinoblastoma treatments include chemotherapy, cold therapy, and laser therapy [2]. Radiation therapy may be another option [2]. Surgery to remove the eye can treat retinoblastoma, but it is only used in certain situations [2].
Which treatment is best depends on several factors: the size and location of the cancer, whether the cancer has spread beyond the eye, your child's overall health, and your own preferences [2].
Chemotherapy
Chemotherapy treats cancer with strong medicines, and it is often the first treatment for retinoblastoma [2]. Other treatments might be needed afterward to kill any cancer cells that remain. There are three ways chemotherapy is given.
Type | How it works | Typical schedule |
Systemic chemotherapy | Medicines given through a vein or in pill form travel throughout the body to kill cancer cells. | Typically monthly for several months [2]. |
Intra-arterial chemotherapy | A thin tube is inserted through the skin into an artery in the groin and advanced up near the eye, where the medicine is released directly. | Typically monthly for a few months [2]. |
Intravitreal chemotherapy | Medicines are injected directly into the eye with a needle, usually after other kinds of chemotherapy. | Used when some cancer remains or when it comes back [2]. |
Cold Therapy (Cryotherapy)
Cold therapy, also called cryotherapy, uses extreme cold to damage cancer cells. It is typically used after chemotherapy to kill any cancer cells that are left [2]. For very small retinoblastomas, cryotherapy might be the only treatment needed [2].
During cryotherapy, a very cold instrument is placed on the eye, causing the nearby cells to freeze. The instrument is then removed, and the cells thaw. This freezing and thawing process is repeated a few times in each session [2].
Laser Therapy
Laser therapy uses a laser light to heat up and damage the cancer cells. The medical term for this procedure is transpupillary thermotherapy [2]. It is typically used after chemotherapy to kill any remaining cancer cells. For very small retinoblastomas, laser therapy might be the only treatment needed. Treatments are typically repeated every few weeks until there are no signs of active cancer in the eye [2].
Radiation Therapy
Radiation therapy treats cancer with powerful energy, and two types are used for retinoblastoma.
Type | How it works | When it is used |
Plaque radiotherapy | A small disk holding radioactive material is stitched into place on the eye and left for a few days, slowly giving off radiation to the cancer [2]. | |
External beam radiation | A machine aims energy beams (X-rays, protons, or other radiation) at the cancer while moving around the child. |
Eye Removal Surgery
When other treatments have not worked, or when the retinoblastoma is too large to be treated by other methods, surgery to remove the eye may be used. In these situations, eye removal may help prevent the spread of cancer to other parts of the body [2].
Eye removal surgery involves three stages. First, the surgeon performs enucleation: disconnecting the muscles and tissue around the eye and removing the eyeball, along with a portion of the optic nerve that extends from the back of the eye into the brain [2]. Immediately after removal, the surgeon places a special ball called an implant in the eye socket, and the muscles that control eye movement are sometimes attached to it. After your child heals, the eye muscles adapt to the implant and it may move just as the natural eye did [2], although the implant cannot see.
Several weeks after surgery, a custom-made artificial eye can be placed over the implant [2]. It is made to match the appearance of your child's healthy eye and sits behind the eyelids. As your child's eye muscles move the implant, it will appear that your child is moving the artificial eye.
Risks of surgery include infection and bleeding [2]. Removing an eye will affect your child's vision, and most children adapt to the vision changes over time [2]. Your child will need to take extra caution to protect the healthy eye, for example by wearing protective glasses or sport goggles while playing sports [2].
Clinical Trials
Clinical trials are studies to test new treatments and new ways of using existing treatments [2]. While clinical trials give your child a chance to try the latest in retinoblastoma treatments, they cannot guarantee a cure [2]. Ask your child's doctor whether your child is eligible to participate, and discuss the benefits and risks of enrolling.
How Can Families Cope With a Retinoblastoma Diagnosis?
When your child is diagnosed with cancer, it is common to feel a range of emotions. Parents sometimes say they felt shock, disbelief, guilt, or anger after their child's diagnosis [2]. Everyone finds their own way of coping. Until you find what works for you, there are several approaches worth trying.
Gather all the information you need. Learn enough about retinoblastoma to feel comfortable making decisions about your child's care. Talk with your child's healthcare team and keep a list of questions for the next appointment. The websites of the National Cancer Institute and the American Cancer Society are good places to start [2].
Organize a support network. Find friends and family who can support you as a caregiver. Loved ones can accompany your child to appointments or sit by the bedside when you cannot be there, and they can spend time with your other children or help around the home.
Take advantage of resources for kids with cancer. Ask your clinic's social workers about special resources for families of children with cancer. Support groups for parents and siblings connect you with people who understand what you are feeling [2]. Your family may be eligible for summer camps, temporary housing, and other support.
Maintain your routine as much as possible. Small children cannot understand what is happening to them during cancer treatment, so keeping a usual routine helps. Arrange appointments so your child has a set nap time [2], keep routine mealtimes, and allow time for play. Bring items from home to the hospital to make your child more comfortable. Hospitals may also have recreation therapists or child life specialists who can share advice and resources.
How Should You Prepare for Your Child's Appointment?
Start by making an appointment with your child's doctor or other healthcare professional if your child has any signs or symptoms that worry you. If an eye problem is suspected, you may be referred to an ophthalmologist, a doctor who diagnoses and treats eye conditions. If retinoblastoma is suspected, your child may see an ocular oncologist, a doctor who specializes in treating eye cancer [2].
Because appointments can be brief, it is a good idea to be prepared. Ask about any pre-appointment restrictions, such as diet limits, when you make the appointment. Write down your child's symptoms, including any that seem unrelated, and key personal information such as major stresses or recent changes in your child's life. Make a list of all medicines, vitamins, and supplements your child is taking. Take a family member or friend along, and write down your questions in order from most to least important [2].
Questions you can ask: What kinds of tests does my child need? What is the best course of action? What are the alternatives to the primary approach you are suggesting? Should my child see a specialist [2]?
What the doctor may ask: Has your child ever had cancer before? Does your family have a history of cancer? Does your child have any siblings, how old are they, and have they ever had an eye exam? When did your child begin experiencing symptoms? Have the symptoms been continuous or occasional? How severe are they? What seems to improve or worsen your child's symptoms? [2]
What is the bottom line on retinoblastoma?
Retinoblastoma is a rare childhood eye cancer, but it comes with a reassuring story: it rarely spreads, especially when found early [1], and for most children treatment does not require removing the eye [1]. The single most powerful thing a family can do is pay attention to a child's eyes, especially a white glow in flash photos, and act quickly on any changes. Families with a history of retinoblastoma have real options, from genetic counseling to eye exams that begin soon after birth [1], that can catch the cancer when it is small and has a greater chance of being cured. If something about your child's eyes concerns you, the best next step is an appointment with your child's doctor.
Talk to your child's doctor about your child's eyes. Whether you noticed a white glow in a flash photo or any change that worries you, an early eye exam is the safest next step. If retinoblastoma runs in your family, ask about genetic counseling and early screening for your children. For more plain-language health guides, visit Rinnit.com.
Frequently Asked Questions
What is retinoblastoma?
Retinoblastoma is a kind of eye cancer that starts as a growth of cells in the retina, the light-sensitive lining on the inside of the eye [1].
Who gets retinoblastoma?
Retinoblastoma happens most often in young children and is usually diagnosed before age 2 [1]. It most often affects one eye, though sometimes it happens in both [1].
What is the first sign parents usually notice?
Often a white color in the center circle of the eye when light is shone in [1], which might show up in flash photos. Other signs include eye redness, eye swelling, eyes that seem to look in different directions, and vision loss [1].
What causes retinoblastoma?
Changes in the DNA inside the cells of the eye make them multiply quickly and survive longer than they should, forming a tumor in the retina. In some children, these DNA changes are inherited from a parent [1].
Can retinoblastoma show up in flash photos?
Retinoblastoma can. A white color in the center of the eye in a flash photo is one of the recognized signs [1], so any concerning eye change deserves a check by your child's doctor.
Does retinoblastoma spread?
Rarely, especially if it is found early. Cancer cells can break away and spread to other parts of the body, which is called metastatic cancer, but retinoblastoma rarely does this [1].
Will my child lose an eye?
Most children do not. For most children, treatment does not require removing the eye [1]. Surgery to remove the eye is used when other treatments have not worked or when the tumor is too large to treat by other methods [2].
Can retinoblastoma be prevented?
There is no way to prevent it. Families with a history of retinoblastoma can use genetic counseling, genetic testing, and eye exams that may begin soon after birth [1], so that any cancer is found when it is small and has a greater chance of being cured.
References
Mayo Clinic Staff. Retinoblastoma - Symptoms and causes. Mayo Clinic. Published March 8, 2024. https://www.mayoclinic.org/diseases-conditions/retinoblastoma/symptoms-causes/syc-20351008
Mayo Clinic Staff. Retinoblastoma - Diagnosis and treatment. Mayo Clinic. Published March 8, 2024. https://www.mayoclinic.org/diseases-conditions/retinoblastoma/diagnosis-treatment/drc-20351013
Shields CL, et al. Retinoblastoma management: achieving high standards of care. Eye (Lond). Published online October 6, 2026. PMID 42839089. https://doi.org/10.1038/s41433-026-04939-7
Sherief ST, et al. Clinicopathological Presentation of Retinoblastoma in Ethiopia. Ocul Oncol Pathol. 2022;8(3):168-174. PMID 36938376. https://doi.org/10.1159/000525113
Gargallo P, et al. Retinoblastoma: towards an earlier diagnosis. Arch Soc Esp Oftalmol (Engl Ed). 2018;93(9):439-443. PMID 29929761. https://doi.org/10.1016/j.oftal.2018.05.003
Bowman R, Arazi M, Cassoux N, et al. Retinoblastoma outcomes in Europe: a prospective analysis of 483 patients from 40 countries. Eye (Lond). 2026;40(3):390-398. PMID 41408393. https://doi.org/10.1038/s41433-025-04144-y
Nishath T, Stacey AW, Steinberg D, et al. Retinoblastoma survival and enucleation outcomes in 41 countries from the African continent. Br J Ophthalmol. 2025;109(1):64-69. PMID 39122353. https://doi.org/10.1136/bjo-2023-324746
Medical disclaimer
This article is for general educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of a qualified healthcare provider with any questions about your child's health. If you notice a white glow in your child's eye or other eye changes, arrange an eye exam promptly.
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