Retinoblastoma factsheet

Introduction

Retinoblastomas are tumours that grow in the back part of the eye, called the retina.

The retina is made up of layers of nerve tissue that are sensitive to light. These nerves convert light through the eye into signals that go to the brain, which tells the brain what you are seeing.

Retinoblastoma is the most common type of eye tumour in children and is usually found before age five.

They can appear in one or both eyes and rarely spread to other body parts.

Signs and symptoms

The most common early sign of retinoblastoma is leukocoria (a cloudy white pupil). In bright light, the pupil can look silvery or yellow.

Other signs include:

strabismus or crossed eyes
a pupil that is bigger than normal
red irritation in the eye that does not go away
poor vision.

Diagnosis

Your child’s local doctor will try to rule out any other illnesses first. They will:

take a blood test
take a medical and family history
do a physical check.

Your child will be referred to a cancer specialist, called an oncologist and an eye specialist, called an ophthalmologist.

To diagnose neuroblastoma, they will:

carefully check the eye by using drops to make the pupil bigger
do scans to see inside the eye, like ultrasound and MRI
do blood tests
do genetic testing.

The oncologist may also do some tests to see if the cancer has spread anywhere else in the body.

Treatment

Treatment options will depend on:

the size of the tumour
whether it is in one or both eyes
whether it has spread to other parts of the body.

Treatments for neuroblastoma can include a combination of:

surgery to remove the tumour
chemotherapy – a drug treatment that uses powerful chemicals to kill fast-growing cancer cells
cryotherapy - a medical procedure that uses very low temperatures to kill cancer cells
light coagulation - a type of therapy where laser beams are used to kill cancer cells
radiotherapy – a therapy where high-energy X-rays are used to kill cancer cells.

Your child will also have regular eye exams to make sure the treatment is working. These are usually done under general anaesthesia, meaning your child will be asleep and not feel any discomfort.

Management

Hereditable retinoblastoma

Some types of retinoblastomas are heritable. This means they can be passed down in families.

Babies who have a parent or sibling with neuroblastoma should have an eye exam shortly after they are born. This is to check for tumours in the eye.

Early detection and treatment of retinoblastoma in babies can mean:

they may need less treatment
there may be a better chance of saving their eyesight.

Children born with hereditable retinoblastoma in one eye have a higher chance of developing it in the other eye as they grow.

They will need regular eye checks with an ophthalmologist after treatment.

Prosthetic eye

Some children with retinoblastoma may need the affected eye removed as part of treatment.

Children who have an eye removed can get an artificial or prosthetic eye that can help:

balance the appearance of their face
make the affected eye socket more comfortable
improve comfort and self-confidence.

Prosthetic eyes are made to suit each child specifically and can look just as natural as the other eye.

Most children will need a new prosthetic eye every few years as they grow and their faces change.

Support for families

A cancer diagnosis can leave families and children feeling overwhelmed, scared, anxious, and upset. Practical and emotional support during and after treatment is essential and can come from:

family
friends
healthcare professionals
specialised support services.

Speak to your child’s treatment team for information about support services.

Retinoblastoma