Retinal cancer (retinoblastoma)


What is it about ?

Retinoblastoma is a rare cancer of the retina (the membrane that covers the back of the eye). As the disease develops in a growing eye, it only affects children. Retinoblastoma starts by the age of 5 at the latest, although it is sometimes not discovered until later. Almost half of the time the disease is inherited. Even if you are the first member of the family to have retinoblastoma, it can still be an inherited form.

In the hereditary form, both eyes are often affected. In the non-hereditary form, only one eye is affected.

Retinoblastoma can make you blind. In extreme cases, the person can even die from it.

Where and how often?

Retinoblastoma can already develop in a child when it is in the mother’s womb. The majority of affected children are under 3 years old. People with hereditary retinoblastoma have a 1 in 2 chance of passing the genetic predisposition on to their children. In addition, retinoblastoma increases the risk of developing other tumors later in life. The most common tumors are: malignant moles (melanoma), bone, muscle and connective tissue tumors, bladder cancer and lung cancer.

How to recognize it?

The most common symptom is a white reflection on the pupil. This can be seen clearly in photos taken with a flash. If there is a white spot on your child’s pupil, urgently go to the ophthalmologist. Squinting is the second most common symptom. This symptom develops even before the appearance of the white reflection.

How is the diagnosis made?

If a white reflection occurs in a child’s eye, the GP will immediately refer you to a specialist, even if their examination cannot yet confirm the diagnosis. A child who gradually starts to squint when he did not squint before is also a reason to see the specialist urgently.

The ophthalmologist will perform various examinations of the eyes to see if a tumor is present and to determine its size. This is an ultrasound examination, a CT scan and an examination of the retina with a special camera. If the ophthalmologist diagnoses retinoblastoma, a blood test will also be taken for DNA analysis.

What can you do ?

See a doctor if you notice a white glint in your child’s eye or if they squint more and more when they see something. Early detection is important to prevent the child from going blind.

If you are planning to become pregnant and your partner has a personal or family history of retinoblastoma, it is best to talk to a doctor. He will refer you to the specialist for additional examinations. Genetic tests can determine if your child is at risk of developing retinoblastoma. Retinoblastoma can be detected by ultrasound several weeks before the due date. In this case, it is possible to induce labor earlier to begin treatment in the baby.

What can your doctor do?

First, if necessary, the doctor will give drugs that reduce the size of the tumor. Then the tumor can be destroyed by laser treatment, cryotherapy or local radiation therapy. Very rarely, general radiation therapy or surgical removal of the eye (called “enucleation”) will be required. DNA analysis is recommended to know for sure whether retinoblastoma is hereditary or not.

Want to know more?

Source

Foreign clinical practice guide ‘Retinoblastoma’ (2000), updated on 04.09.2017 and adapted to the Belgian context on 08.01.2020 – ebpracticenet