Treating retinoblastoma

Retinoblastoma is a rare cancer. Your child will go to a specialist centre for treatment.

There are different treatments such as:

• freezing the cancer (cryotherapy)
• laser therapy
• chemotherapy
• radiotherapy
• surgery

The treatment your child has depends on several things. This may include the size of the cancer and if it is in one or both eyes.

Treatment centres

There are 2 specialist retinoblastoma centres in England. They are:

• The Royal London Hospital

• Birmingham Children's Hospital

These 2 centres serve all parts of the UK including Wales, Scotland and Northern Ireland. They have teams of specialists who know a lot about retinoblastoma and how to treat it.

There is a lot to think about as a parent and you’re likely to worry about getting the best treatment for your child. Talk to the doctors or specialist nurses about any worries that you have. They might also be able to put you in touch with other parents of children who have already been through diagnosis and treatment.

Deciding which treatment your child needs

A team of doctors and other professionals discuss the best treatment and care for your child. They are called a multidisciplinary team (MDT).

Treatment aims to:

• get rid of the cancer
• save the eye or eyes
• provide the best possible vision for your child

The doctors also want to provide treatment with the fewest possible side effects. This is for both in the short term and in the long term. 

Treatment planning for your child depends on several factors including:

• where the cancer is

• if the cancer is in one or both eyes

• the size of the cancer

• how far it has spread

• the number of sites of cancer in the eye

Your child’s doctor will talk to you about the treatment, its benefits and the possible side effects.

Types of treatment:

Children usually have their treatment while they are asleep. This means they have it under a general anaesthetic . They’ll be awake if they’re having chemotherapy into their bloodstream. This is called systemic chemotherapy.

Freezing therapy (cryotherapy)

Cryotherapy is a treatment that uses extreme cold to destroy cancer cells. It’s a local treatment, which means that it only affects the area where you have the treatment. It doesn’t treat any cancer cells in other parts of the body. Cryotherapy is best for very low and some low risk retinoblastomas in the outer edge of the retina.

The doctor uses a metal probe that’s cooled to a very low temperature. The probe then freezes the cancer cells and kills them.

Laser therapy

There are 2 types of laser treatment for retinoblastoma:

• photocoagulation

• thermotherapy, this is also called transpupillary thermal therapy (TTT) or heat therapy

A laser is a very thin, focused beam of light. It destroys the tissue it’s directed at. The doctor aims the beam through the dark centre of the eye called the pupil. 

Photocoagulation laser treatment is different to thermotherapy. This is because the laser delivers the light at different strengths. Photocoagulation laser treatment can help to destroy the blood vessels that feed the cancer.

Thermotherapy is another type of laser treatment. This treatment uses infrared light. The doctor aims this light at the cancer and slowly burns away the cancer cells, destroying them by heat.

Your child usually has laser treatment if the cancer is small and at the back of the eye. They might also have it with or after chemotherapy.

Radiotherapy

Doctors treat some retinoblastomas with radiotherapy. Radiotherapy means the use of radiation to treat cancer cells.

Brachytherapy

Your child might have a type of radiotherapy called brachytherapy . This means your child has a small radioactive disc called a plaque stitched directly over the cancer. The plaque works by continuously delivering a high amount of radiation to the small part of the eye it covers. You might hear it called localised radiotherapy. Your child will have the plaque in place for a few days. The doctor then removes the plaque.

Proton beam radiotherapy

Proton beam radiotherapy is a type of external radiotherapy. Your child might have this if the cancer has spread to the:

• nerve between the eye and the brain (optic nerve)
• hole in the skull where the eye sits (eye socket)

Proton beam therapy uses a different type of radiation to brachytherapy. It is called proton radiation. The proton beams are aimed precisely at the cancer. This type of radiation causes less damage to the healthy tissue around the eye.

We have more information about radiotherapy to the eye area. It is for adults with eye cancer.

Surgery

Your child might need surgery to remove the eye. This is called an enucleation. This is usually when the cancer is large and the eye is no longer working. If your child has cancer in both eyes, they might remove the worst affected eye. 

It can be devastating to find out your child needs to have their eye removed. Your child will usually recover and adapt quickly. And it doesn’t usually stop them from doing what they want to do.

After removal of the affected eye the surgeon fits an implant. This is like a small ball to replace the lost eye. This will fill their eye socket. It’s attached to the muscles, so it moves in sync with the other eye.

Your child also has an artificial eye (prosthesis) made to cover the implant. This will match their original eye and is like a large contact lens. Your child usually has this fitted a few weeks after surgery.

We have more information about surgery to the eye. It is for adults with eye cancer.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. Your child might have their chemotherapy in one or more of the following ways:

• into their bloodstream (intravenously) through a long flexible plastic tube - systemic chemotherapy

• through a blood vessel (artery) to the affected eye - intra arterial chemotherapy or IAC

• as an injection directly into the jelly part of the eye - intravitreous chemotherapy

Your child might have chemotherapy:

• to try and shrink the cancer first if it is large. They might have this before a local treatment such as laser therapy
• if there is any risk that the cancer might have spread

They usually have a combination of chemotherapy drugs. Doctors commonly use a combination called CEV also known as JOE. This includes:

• carboplatin

• etoposide

• vincristine

This combination is given in cycles  of treatment.

Other chemotherapy drugs used for systemic chemotherapy include:

• cyclophosphamide

• ifosfamide

• doxorubicin

• topotecan

Doctors use the following chemotherapy drugs for treatment through a blood vessel or as an injection directly into the jelly part of the eye:

• melphalan

• carboplatin

• topotecan

Side effects

The side effects of having chemotherapy into the artery or vitreous of the eye can be different to those of intravenous chemotherapy. These might include side effects such as:

• inflammation
• a droopy eyelid
• a squint

These are usually temporary. There can also be a risk of reduced vision.

Your child’s doctor will go over the possible risks of having chemotherapy into the eye with you.

Going to sleep (general anaesthetic)

Your child will receive most of their treatment, tests or scan while they are asleep. They won't be asleep when having systemic chemotherapy.

To get your child to sleep a nurse or doctor puts a small tube (cannula) into your child’s hand or arm. The nurse first puts some local anaesthetic cream on the area to numb it. Your child might still feel the needle go in, but it shouldn’t be painful.

You can usually go with your child to theatre before the operation or examination. You wait with your child in a room next to the theatre, called the anaesthetic room. You can give your child a cuddle and reassure them until they are beginning to fall asleep.

The anaesthetist gives your child the anaesthetic through the cannula in their hand. Or, they gently place a mask over your child’s mouth. Your child breathes in the anaesthetic gas until they fall asleep. A member of the team will let you know when the treatment, test or scan has finished.

It’s not unusual to feel very emotional seeing your child drift off to sleep. The theatre or ward team may take your mobile number and call you as soon as they are awake.

Side effects

Your child’s doctor will talk to you about the possible side effects. The side effects your child might have depend on the type of treatment they are having. Some of the possible side effects include:

Loss or changes to eyesight

Most parents worry about whether their child will lose their sight. Whenever possible, the doctor will try to keep the sight of the eye. Removing the affected eye will not cause sight loss in the other eye. Children learn to adapt very quickly and live a normal life, often going to mainstream school.

If your child has retinoblastoma in both eyes, it’s possible that your child will lose some sight. This might mean they need to have visual impairment support. This will be either in a mainstream school or a specialist school.

Some treatments, such as laser treatment, might cause changes to their sight. Your child might complain that it’s not as bright as before. Other comments might be that they can see dots or lines (floaters) and flashes of light. Let their team know straight away if this happens.

Increased risk of infection

Chemotherapy can cause a drop in your child’s white blood cells. Symptoms include:

• a change in temperature
• aching muscles
• headaches
• feeling cold and shivery and generally unwell

Your child might have other symptoms depending on where the infection is.

You should contact your child’s advice line urgently if you think they have an infection. 

Hearing changes

Some chemotherapy drugs can cause changes to your child's hearing. These include carboplatin and cisplatin. They might have hearing tests before these types of drugs. Let your child’s doctor or nurse know if you or your child notices anything different with their hearing.

A second cancer

Although the risk is very small, chemotherapy can cause a second cancer to develop later in life. If your child has an inherited retinoblastoma, they are more at risk of getting a second cancer later on. These cancers include sarcoma or melanoma. Your child’s doctor will talk this through with you and answer any questions you may have about it.

Trilateral retinoblastoma is a very rare type of cancer. Children with inherited retinoblastoma can develop this. It starts from the pineal gland in the centre of the brain, behind the brain stem. It makes the hormone melatonin which controls sleep. Symptoms include headaches and feeling and being sick.

If your child is at risk of this, your doctor will talk to you about it. Your child will have regular follow ups.

Retinoblastoma that has come back or not gone away

Almost all retinoblastomas are successfully treated. But some cancers can come back again (recurrence or relapse) or don’t go away (refractory). Your child’s doctors will develop a treatment plan. This will depend on:

• what treatment they have already had

• where the cancer is

• the size of the cancer

• how far it has spread

• your child’s general health

Your child’s healthcare team will explain the different treatment options. They will support you through this difficult time.

Follow up

Your child will have regular follow up appointments with their specialist. They are very close together to start with and then get gradually further apart as time goes on. These appointments are to check:

• how they are recovering

• their development

• if they are having any problems following treatment

• if there are any signs the retinoblastoma has come back

These appointments usually include your child having a general anaesthetic. This way their doctor can have a close look at their eyes.

Your child will have genetic counselling when they are old enough to understand their condition. They will also have advice on living a healthy lifestyle. This can help them reduce their risk of getting a second cancer by:

• avoiding smoking

• protecting their skin from the sun

• following a healthy diet

• doing exercise

• using alcohol moderately

Coping when your child has cancer

It is understandably devastating when a child is diagnosed with cancer. Although many children with cancer are successfully treated, it is a difficult time for everyone involved.

You and your child will also have support from several professionals during and after treatment. These include:

• doctors and nurses on the ward
• your GP
• cancer nurse specialists
• social workers
• play specialists
• a psychologist or psychotherapist

Talking to other people who are experiencing the same thing can also help. Our online discussion forum, Cancer Chat, is a place for anyone affected by cancer. You can share experiences, stories and information with them.

There are children's cancer organisations that you can contact for help and support.

The Childhood Eye Cancer Trust is a charity. They support families and people affected by retinoblastoma. It:

• offers support and information
• funds research
• raises public awareness of this rare cancer

Tel: 020 7377 5578 (Monday to Friday, 9am to 5pm)
Email: info@chect.org.uk