Retinoblastoma: Understanding this Rare Eye Cancer

Cancer can affect all parts of the body, but when it occurs in the retina, or the layer of nerve cells lining the back of the eye, it leads to retinoblastoma. This rare condition is the most common type of eye cancer occurring in children, with 200 to 300 children diagnosed each year, according to the American Cancer Society.

Read on to familiarize yourself with the symptoms and types of retinoblastoma, as well as common treatment options.  

Retinoblastoma Symptoms
Because retinoblastoma is seen more commonly in children than adults, with the average age of diagnosis being 2 years old, parents and caregivers need to learn how to recognize the symptoms of this condition. Children might struggle to put their feelings into meaningful words at this age, which could delay necessary medical treatment. Early detection can help improve the likelihood of a good outcome.

Here are some symptoms to watch for if your child complains of eye issues:

Changes to Retina Appearance
If you’ve had pets in your family, you’ve likely noticed it can be challenging to take a good photo of them with the flash on. In some animals, eyeshine is a naturally occurring phenomenon that happens when light bounces off a reflective layer behind the retina. 

If a child’s eye reflects white, silvery, grey or yellowish instead of red in low light or in a photograph, it could be a sign of leukocoria, according to the Cleveland Clinic. This occurs when something gets in the way of light reflecting naturally off the retina, causing a noticeable color difference that fills most or all of the pupil. 

Eye Movement Issues
Eyes that don’t appear to look in the same direction, a condition commonly known as lazy eye or strabismus, or have trouble following movement can signal underlying eye issues. While lazy eye, in particular, is often caused by mild muscle weakness, retinoblastoma can also cause this condition. 

Other signs and symptoms include:

·         Vision problems

·         Eye pain

·         Redness in the sclera, or the white part of the eye

·         Bleeding between the cornea and iris, also known as hyphema

·         Eye bulging, or proptosis

·         Pupil doesn’t react when exposed to bright light

·         Different colors appearing in each iris

Retinoblastoma is typically confirmed with imaging technology, such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) or positron emission tomography (PET) scans. 

What Causes Retinoblastoma?
During the early stages of gestational development, the eyes have cells known as retinoblasts. Their job is to make new cells that fill the retina until they stop and become mature retinal cells.

However, if there is a problem with the RB1 gene, a tumor suppressor gene responsible for normal tissue development, the retinoblasts will continue to grow instead of maturing.

Retinoblastoma is categorized based on whether it affects one or both eyes:

·         Unilateral retinoblastoma – Affecting only one eye, this is considered the most common of the two types. Genetic errors present only in the tumor cause most unilateral cases; this is also known as the sporadic form, meaning there is no risk to other family members.

·         Bilateral retinoblastoma – Almost always hereditary, this form affects both eyes. Children with this form of retinoblastoma are more likely to develop other tumors and second cancers after radiation exposure; they are also at risk of passing the condition to their children. 

Retinoblastoma Treatments
There are a number of treatment options available to discuss with a doctor, with some used in combination with each other:

·         Chemotherapy – Doctors may use chemotherapy to attack cancerous cells directly. Chemotherapy can help avoid complications with surgeries that could cause vision loss. They can also shrink tumors so they can be destroyed through other treatments. 

·         Radiation therapy – High-frequency energy or particles can kill cancer cells through two treatments known as external beam radiation therapy (EBRT) and intensity modulated radiation therapy (IMRT). EBRT focuses radiation beams from outside the body on the cancer; IMRT works similarly, but doctors can heighten the intensity to deliver higher doses.

·         Focal therapy – Whether cryotherapy, thermotherapy or laser therapy, doctors use these treatments to “focus” heat, extreme cold or lasers at cancerous cells and destroy them. These methods can also target the small veins feeding tumors. 

·         Surgery – If there’s a risk the retinoblastoma will spread to other parts of the body, doctors might turn to surgery to prevent it. If a doctor suggests enucleation, or removing the affected eye, it’s likely the affected person has already experienced vision loss. 

While retinoblastomas are usually seen in children, it is not impossible to experience one as an adult. Maintaining good eye health with regular visits to an ophthalmologist is the best way to catch this disease early and ensure your family has access to a wide array of treatment options. To get more valuable eye health insights, visit healthyvisionassociation.com/articles. 

Articles in this newsletter are supposed to be informative, enlightening and helpful to you. While all information contained herein is meant to be completely factual, it is always subject to change. Articles are not intended to provide medical advice, diagnosis or treatment. Consult your doctor regarding any concerns you have.