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Retinoblastoma Program

The experts within the Retinoblastoma Program at Phoenix Children’s Hospital provide lifesaving and eye-saving treatments for your child.

Retinoblastoma is a rare condition. Because of this, there are a limited number of doctors trained to treat this type of pediatric eye cancer.

At Phoenix Children’s Hospital, our Retinoblastoma Program combines the expertise of many specialists to diagnose and treat this serious condition. We’re focused on offering lifesaving and eye-saving treatments for your child.

In addition to providing care for retinoblastoma, our team treats children with other cancer-related eye conditions and noncancerous eye tumors. These include:

  • Conjunctival nevus: A mole-type growth on the white part of the eye
  • Iris cysts: Benign (noncancerous) fluid-filled cysts in the iris (the colored part of the eye) that can disrupt vision
  • Neurofibromatosis: A multiorgan disease that can cause a tumor on the eye called optic nerve glioma
  • Medulloepithelioma: A rare tumor in the middle layer of the eye (uvea)
  • Systemic cancers: Cancers, like leukemia and lymphoma, that spread to the eyes 
  • Tuberous sclerosis: A disorder that can cause benign tumors, called astrocytomas, in the retina that can cause vision changes
  • Von Hippel Lindau syndrome: A disorder that causes benign tumors, called capillary hemangiomas, in the eye, which can lead to detachment of the retina and permanent vision changes

What Is Retinoblastoma?

Retinoblastoma is a tumor in the eye’s retina. The retina is the back part of the eye that helps you detect light and color.

Retinoblastoma, though rare, is the most common type of eye cancer in children. The disease typically affects children under the age of 5. Diagnosis for most children with retinoblastoma occurs between 12 and 18 months of age.

Without proper treatment, retinoblastoma can lead to blindness. Advanced retinoblastoma can also spread cancer to other parts of the body.

What Causes Retinoblastoma?

Retinoblastoma is caused by a change in a gene during eye development. This gene change causes retina cells to grow uncontrollably.

While some children who get retinoblastoma have a family history of the disease, most do not.

For the majority of children with retinoblastoma, only one eye is affected. Children with this type of retinoblastoma, called “sporadic,” are not at increased risk for other cancers.

In a smaller number of children with retinoblastoma, the gene change affecting retina cells is present in cells throughout the body. For these children, retinoblastoma presents at an earlier age. In about a quarter of these cases, retinoblastoma affects both eyes. Children with this form of retinoblastoma are also at increased risk for other cancers.

What Are the Symptoms of Retinoblastoma?

A common symptom of retinoblastoma is a white glow or reflection in the pupil. Parents of children with retinoblastoma are often the first to notice this symptom because it shows up in flash photography.

Children with retinoblastoma can also develop strabismus (lazy eye).

Other less common symptoms of retinoblastoma include:

  • Eye bulging
  • Eye pain, redness or swelling
  • A pupil or pupils that don’t change in size when exposed to light
  • Vision changes

Because the symptoms of retinoblastoma are similar to symptoms of other conditions, it’s important to visit your health care provider for the right diagnosis.

How Is Retinoblastoma Diagnosed?

If your child’s doctor or ophthalmologist suspects retinoblastoma, they will refer you to an ocular oncologist. Ocular oncologists specialize in treating eye cancers.

Your child’s ocular oncologist will perform tests to confirm a retinoblastoma diagnosis.

Examining the eyes of small children is tough to do. Because of this, some tests for retinoblastoma require your child to have anesthesia.

Your doctor at Phoenix Children’s will work alongside experts from our Child Life Program to help your child with any fears they may have about tests or procedures.

Diagnostic tests for retinoblastoma include:

  • Ocular ultrasound. This test uses sound waves to view the eyes. 
  • Fluorescein angiography. This test uses a fluorescent dye to look at blood vessels in the eye.
  • Optical coherence tomography (OCT) scan. This test uses a special light to scan the retina layer by layer to look for changes.
  • Magnetic resonance imaging (MRI). In rare cases, a brain tumor can be associated with your child’s retinoblastoma. Your doctor will order an MRI at the time of diagnosis. MRIs use large magnets, radio waves and a computer to make detailed images.

How Is Retinoblastoma Treated?

Each child with retinoblastoma requires a different treatment approach. In many cases, multiple treatments are combined. Treatments include:

  • Chemotherapy. Your ocular oncologist and pediatric oncologist may recommend chemotherapy. Chemotherapy is a type of medication delivered via the bloodstream (systemic chemotherapy) or directed in to the artery supplying the eye (intra-arterial) or directly into the eye (intravitreal). Chemotherapy works by killing cancerous tumor cells.
  • Cryotherapy (freezing). During cryotherapy, doctors freeze tumor tissue to encourage shrinking. Cryotherapy is often combined with chemotherapy.
  • Laser therapy. During this treatment, a laser is used to shrink the retinoblastoma tumor.
  • Radiation therapy. Radiation therapy uses radiation to kill cancer cells. Radiation can be delivered internally via a custom-made disc that fits on your child’s eye. Radiation can also be delivered by a machine that sends a beam of radiation to your child’s eye.
  • Surgery. In more advanced cases of retinoblastoma, removal of the eye is required. Surgery to remove the eye would be recommended to prevent the spread of retinoblastoma to the brain or other parts of the body.

Genetic Testing and Counseling

If your child is diagnosed with retinoblastoma, our team at Phoenix Children’s will recommend genetic testing. This testing will determine if their retinoblastoma is inherited.

It’s important to know if retinoblastoma is inherited. If your child’s retinoblastoma is inherited, their siblings and future children are at risk for developing the disease.

The Genetic Counseling team at Phoenix Children’s will advise you on recommended screenings for your family.

What to Expect at Phoenix Children’s

Kids are our focus at Phoenix Children’s. Here, you and your child will be met with a compassionate team of experts — all trained to care for childhood disease.

Our ophthalmology RN Kimberly coordinates care for all our ocular oncology patients. She is your first point of contact for referrals and clinical questions. She completes intake from outside referring facilities, ensures all treatments and results are received at proper intervals, and assists in letter writing including for charitable flight organizations.  

Our surgery scheduler Kristy schedules all exams under anesthesia, while working with other departments to combine procedures while the patients are under anesthesia. She arranges Ronald McDonald House accommodations for out of state patients and for Arizona families that live greater than 30 miles from the hospital. 

Ronald McDonald House Charities of Central and Northern Arizona offers two nearby Ronald McDonald House locations for the families of children in our care. To be eligible to stay, you must live more than 30 miles from Phoenix Children’s. 

Because we are one of only a few pediatric hospitals in the U.S. equipped to comprehensively treat retinoblastoma, many of our patients travel for care.

Phoenix Children’s Hospital works with charitable flight organizations for out of state patients. Air Care Alliance has general information and contact information for these organizations. Families have an application process to see if they are eligible. If eligible Kimberly provides required documentation of upcoming treatment to families and these flight organizations.

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