Pediatric Retinoblastoma

Retinoblastoma is the most common eye tumor in children. If left untreated, the tumors can grow and fill most of the eye. At Children's Health, we offer advanced diagnostic testing and therapies, which helps us determine a personalized treatment plan for your child. It also gives us the best chance of eliminating their cancer and preserving their vision.

What is Pediatric Retinoblastoma?

Retinoblastoma is a tumor that begins in the retina, the thin layer of tissue on the back of the eye. The cancer almost always begins with a change in the retinoblastoma (RB1) gene. Normally, this gene helps to keep cells from growing out of control, but a combination of factors can cause the gene to change and stop working correctly.

What are the different types of Pediatric Retinoblastoma?


This is where a child only has retinoblastoma in one eye.


Hereditary retinoblastoma means a child has a genetic predisposition to developing retinoblastoma and usually has retinoblastoma in both eyes.

What are the signs and symptoms of Pediatric Retinoblastoma?

Many parents notice these tumors when they take a picture of their child with a flash and their child’s pupil is white in the photo. Other signs and symptoms include:

  • Lazy eye
  • Pain in the eye
  • Bleeding in the front part of the eye
  • Bulging eye
  • Redness in the sclera (the white part of the eye)
  • A pupil (the dark part of the eye) that stays the same size when exposed to bright light
  • A different color in the iris (the colored part of the eye)

How is Pediatric Retinoblastoma diagnosed?

Parents typically take their kids to eye doctors after they notice the above symptoms. The eye doctor can confirm it’s a tumor. After diagnosis, our main goal is to preserve vision and save the child’s life.

What causes Pediatric Retinoblastoma?

This cancer happens because of a mutation in the retinoblastoma (RB1) gene. Approximately 40% of the time, the child’s genes develop this mutation very early during pregnancy or the child inherits the mutated gene from a parent. The remaining 60% of the time, the mutations spontaneously happen in the eye after birth.

How is Pediatric Retinoblastoma treated?

Our team typically uses a combination of surgery, laser therapy, cryotherapy and chemotherapy to treat these cancers. Their treatment will depend on the type and severity of cancer your child has.

We offer advanced genetic testing that looks at a possible mutation in the retinoblastoma (RB1) gene. If we find out your child has an inherited mutation in this gene, this can make a huge difference in creating their treatment plan, preserving vision and curing the retinoblastoma.

If left untreated, retinoblastoma tumors can grow, resulting in blindness and ultimately, death.

Treating Sporadic Retinoblastoma

Typically, we treat sporadic retinoblastoma with surgery or a combination of surgery and chemotherapy. Many times, our surgeons have to remove the entire eye to keep the cancer from spreading.

Hearing that your child has to have surgery to remove their eye is very difficult, but doing the surgery as soon as possible gives kids the best chance of beating their cancer. The ophthalmologist removes the tumor and works with an ocular prosthetic specialist, who creates an artificial eye that replaces the eye that was removed. Our prosthetic specialist is a gifted artist. It's very difficult to see the difference between a child’s natural eye and the new artificial eye.

After your child’s surgery, a specialist will closely examine the tumor under a microscope to see if the tumor has moved into the eye’s tissue. If the tumor hasn’t moved deeper into the eye’s structure, it’s considered to be low-risk and no further treatment is needed. However, if the tumor has spread further into the eye, kids usually need chemotherapy treatments to make sure the cancer doesn’t spread to the brain or other parts of the body.

Treating Congenital Retinoblastoma

Children who have congenital retinoblastoma will need more intensive treatments. Our goal in treating these kids is to take out the tumor while preserving functional vision. We typically use a combination of the following therapies:

  • Cryotherapy: While your child is under general anesthesia, we place a small metal probe in the eye (cooled to a low temperature), which eliminates the retinoblastoma cells by freezing them.
  • Laser therapy: While your child is under general anesthesia, we use a highly focused beam of light that can heat and destroy the retinoblastoma tumor.
  • Chemotherapy: Depending on your child’s diagnosis, they might need multiple rounds of chemotherapy. These are drugs that can destroy cancer cells.


Pediatric Retinoblastoma Doctors and Providers

At Children’s Health℠, our affiliation with UT Southwestern means that you and your child have access to some of the world’s top pediatric cancer specialists. The team consists of hematologists, oncologists and neurosurgeons, who all work together to evaluate your child and develop a comprehensive treatment plan.

Frequently Asked Questions

  • What are the first signs of eye cancer?

    The first time many parents notice retinoblastoma is when they take a picture of their child with a flash and their child’s pupil is white in the photo.

  • How is retinoblastoma inherited?

    We know about 40% of young children with retinoblastoma will have some genetic predisposition to developing retinoblastoma.

  • At what age is retinoblastoma diagnosis?

    Retinoblastoma is typically diagnosed at an early age, from newborns to 4-year-olds.