The main treatments for eye cancer are surgery, radiotherapy and chemotherapy. But you may have other treatments that your eye specialist will discuss with you. Your treatment will depend on:
the type of eye cancer you have and where it is in the eye
the size of your cancer and how far it has grown or spread (the stage)
your general health and level of fitness
As with many types of cancer, the earlier your cancer is diagnosed, the easier it is to treat and possibly cure it. When planning your treatment your doctors will try their hardest to save as much of your sight as they can.
There are different types of eye cancer, depending on the type of cell the cancer starts in. For example, melanoma of the eye and lymphoma of the eye.
Read more about the types of eye cancer
Melanoma of the eyeball
Treatment for eye melanoma is usually surgery or radiotherapy, or both.
Whether you have surgery or radiotherapy depends on:
where the cancer is
the size of the cancer
how much it is affecting your sight
If the cancer is large or already stopping you from seeing out of the eye, you will probably have surgery to remove the eye. This operation is called an enucleation. Otherwise your doctor might recommend that you have one of the following:
radiotherapy
an operation to just remove the cancer (but not the eye)
laser treatment
Ask your doctor as many questions as you need to. Together you can decide which treatment is best for you.
It can come as quite a shock if you need to have surgery to remove your eye. You will need time to come to terms with this change. As long as you have sight in one eye, it may not make much difference to what you can and can’t do. You may still be able to drive, for instance.
It may help to know that many people who have had this surgery rate their quality of life as highly as people who have kept their eye.
Melanoma of the iris
This type of cancer can be so slow growing that you don’t always need treatment, especially if you don’t have symptoms. Your doctor will give you regular check ups to make sure the cancer is not getting bigger.
If the cancer grows, or if it is causing symptoms, you may have one of the following:
removal of the melanoma
removal of the whole eye (an enucleation)
radiotherapy
Choroid or ciliary body melanoma
If melanoma of the choroid or ciliary body is not getting bigger you may not need treatment straight away. You will have regular check ups to make sure the tumour has not started to grow. This is sometimes called periodic observation.
If you do need treatment you might have one of the following:
radiotherapy
photocoagulation
transpupillary thermotherapy
photodynamic therapy
surgery to remove just the cancer
surgery to remove the whole eye (enucleation)
Photocoagulation, transpupillary thermography and photodynamic therapy are all types of laser therapy. They use a source of heat or light to destroy the cancer cells.
Your eye specialist will discuss the treatment choices with you. This usually depends on the size of the cancer.
00CreativarianCreativarian2022-12-07 17:40:492022-12-07 17:40:49Treatment Decisions for Eye Cancer
Retinoblastoma affecting one eye is called unilateral retinoblastoma. Bilateral retinoblastoma is when both eyes are affected.
Who gets retinoblastoma?
Retinoblastoma most commonly affects children under the age of 5. Around 36 children are diagnosed with retinoblastoma in England every year.
Children with retinoblastoma in both eyes are usually diagnosed in the first year of life. Those with one eye affected tend to be diagnosed a bit later, often between 2 and 3 years.
Although the diagnosis can be very distressing and frightening for the child and their parents it has the highest survival of any children’s cancer in England. Almost all children (99-100%) survive for 5 years or more after they are diagnosed.
Causes and screening
Some children are born with a change (mutation) in the retinoblastoma gene that they inherited from one of their parents. Or this gene change happened during the very early stages of their development in the womb. This gene is known as the RB1 gene.
About 4 out of 10 children diagnosed (about 40%) have this heritable (genetic ) type, which often affects both eyes. Heritable retinoblastoma also tends to have more than one tumour in one or both eyes.
The remaining 60% of retinoblastomas are non heritable (non genetic) type. These nearly always affect only one eye. In these the mutation in the RB1 gene occurs later in development and only affects a single cell in the retina to cause a retinoblastoma.
Screening
The aim of screening for retinoblastoma is to spot cancer at an early stage. This means treatment is more likely to be successful and improve your child’s chance of survival.
Who should be checked?
Your child should be checked if they have a close relative such as a parent, brother or sister who has or has had retinoblastoma. This involves regular eye examinations under a general anaesthetic (so your child is asleep). How often and how long your child has screening for depends on their level of risk. They usually have regular screening from birth to the age of 5 years. This may vary between different retinoblastoma centres.
Some children may also have a blood test to check for the RB1 gene. This is only possible if the family member who has had retinoblastoma can be tested first.
If your child’s relative does not have the mutation, they won’t need screening for retinoblastoma.
Talk to your GP if you have a family history of retinoblastoma. They can refer your child to one of the two specialist retinoblastoma centres (Royal London Hospital or Birmingham Children’s Hospital). Your child is unlikely to need screening if a more distant relative, such as a cousin, has retinoblastoma. But the retinoblastoma centre can explain which of the family need screening.
The eye
To understand how retinoblastoma develops, it helps to know a bit about the eyes and how they work. There are 3 main parts to the eye:
the globe (eyeball)
the orbit, which is the bony hollow structure that holds the eye
the accessory structures (or adnexal structures). These protect and support the working of the eye such as the tear glands and eyelids.
The eyeball is filled with a jelly-like substance called vitreous humour. It has a lens at the front. In front of the lens is the coloured part of the eye, called the iris.
The lens and iris focus the incoming light onto the back part of the eye, called the retina. The retina is like the film in the camera. When light hits the retina it transmits a message to the brain via the optic nerve. This allows us to see.
How retinoblastoma develops
Retinoblastoma is a cancer of the retina. When a baby is growing in the womb, the eyes are one of the first things to develop. In the very early stages the eyes have cells called retinoblasts that grow very fast. Later, they stop growing and develop into mature retinal cells that can detect light.
Very rarely, the immature retinoblasts continue to grow very fast and do not turn into mature retinal cells. Instead, they grow out of control and develop into a cancerous tumour called retinoblastoma.
If the tumour is not treated, the cells continue to grow and the cancer fills most of the eyeball. It can also spread to other parts of the eye and begin to block the flow of fluid inside the eye. This leads to a build up of pressure and can cause loss of vision.
Most retinoblastomas are found early and successfully treated before they spread outside the eyeball. If they do spread, they can go to anywhere in the body including the brain, bones and lymph nodes . They can be difficult to treat once they have spread.
Symptoms
Most children with retinoblastoma seem well. But two common signs that parents first notice in their child are that:
they have a white glow or white reflection in the centre of their eye (pupil) – this is also known as leukocoria
their eyes are not looking in the same direction (a squint) – also known as strabismus
The pupil might look white, like a cat’s eye that is reflecting light. Sometimes this is noticed on photos when a flash is used and the pupil appears white rather than the typical red colour.
Other less common symptoms might include:
you or the school noticing your child can’t see properly
inflammation and reddening of their eye
uncontrollable movement of their eye from side to side (nystagmus)
Children do not usually complain of any pain.
In a very few cases, a child who is not gaining weight or developing normally might be referred to a specialist children’s doctor (paediatrician). During their investigations, the abnormal retinoblastoma gene shows up in a blood test.
Urgent referral to a specialist
There are guidelines to help GPs know when to refer a child urgently to see a specialist. This is to help investigate their symptoms further and help rule out cancer.
Some of the UK nations have targets around how quickly they’ll be seen. In England an urgent referral means that your child should see a specialist within 2 weeks.
This 2 week time frame is not part of the waiting time targets for Scotland, Wales and Northern Ireland. But wherever your child lives, they will be seen as quickly as possible.
Ask your GP when they are likely to get an appointment to see the specialist.
Getting diagnosed
Your child will need a number of tests if their doctor suspects they have retinoblastoma. These aren’t generally painful. But your child will have to keep still for some of them.
The team caring for your child are used to helping children have these tests. They will do everything they can to prepare them, and you. For some types of test, your child might need to be drowsy (sedation ) or asleep (general anaesthetic ).
Some of the tests for retinoblastoma include:
examination of the eye using a light and magnifying glass (ophthalmoscopy)
ultrasound of the eye. A wand like probe glides over gel that is placed on the outside of your child’s eyelid. The probe uses sounds waves to form a picture of the eye and its structures on the screen
magnetic resonance imaging (MRI scan). The MRI scan creates a picture using magnetism and radio waves
testing for changes to your child’s genes (genetic testing). This is either a blood test or they use a sample of tissue from the eye. Your child might also have other blood tests to check how well their parts of their body are working, such as the kidneys
The stage of a cancer tells you how big it is and whether it has spread. How retinoblastoma is treated depends on the stage of the tumour.
Retinoblastoma has 2 main stages:
Intraocular retinoblastoma
The cancer is entirely within the eye and has not spread. Your child’s specialist puts the tumour more accurately into one of several more accurate subgroups. These groups are labelled A to E. A being the very low risk group, E being the highest risk group.
Most children are diagnosed with intraocular disease.
Extraocular retinoblastoma
The cancer has spread beyond the eye and into the tissues surrounding it. It might have spread to another part of the body. In the UK this is rare.
Treatment
There are different treatments available for retinoblastoma. The treatment your child has will depend on several factors such as their stage and risk group.
Treatment for retinoblastoma might include one or more of the following:
freezing therapy (cryotherapy)
laser therapy
chemotherapy
radiotherapy
surgery
00CreativarianCreativarian2022-12-07 17:36:222022-12-07 17:36:22What Is Retinoblastoma?
Retinoblastoma is rare and so your child will go to a specialist centre for treatment. There are different treatments such as freezing and laser therapy, chemotherapy, radiotherapy and surgery. The treatment your child has depends on several things including the size of the cancer and if it is in one or both eyes.
Treatment centres
There are 2 specialist retinoblastoma centres in England and 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. Your child may have some of their treatment in their local cancer unit which is overseen by one of the specialist retinoblastoma centres.
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).
The aim of the treatment is to get rid of the cancer, save the eye or eyes, and provide the best possible vision for your child. The doctors also want to provide treatment with the least possible side effects, both in the short and 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.
Treatment overview
Treatment for your child might include one or more of the following:
freezing therapy (cryotherapy)
laser therapy
chemotherapy
radiotherapy
surgery
Children usually have their treatment while they are asleep under general anaesthetic . They’ll be awake if they’re having chemotherapy into their bloodstream (systemic chemotherapy).
Types of treatment:
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 retinoblastomas that are in the outer edge of the retina.
The doctor uses a metal probe that’s cooled to an extremely 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 (transpupillary thermal therapy (TTT)) or heat therapy
A laser is a very thin, focused beam of light that 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 destroys tissues.
Thermotherapy is another type of laser treatment. This treatment uses an 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 also might have laser treatment with or after chemotherapy.
Radiotherapy
Doctors treat some retinoblastomas with radiotherapy. Radiotherapy means the use of radiation to treat cancer cells.
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 continually delivering a high amount of radiation to a small area (the part of the eye it covers). You might hear it called localised radiotherapy. Your child will have the plaque in place for a set number of days. The doctor then removes the plaque.
Proton beam radiotherapy is a type of external radiotherapy. Your child might have this if the cancer has spread to the optic nerve in the socket of the eye. Proton beam therapy uses a different type of radiation to brachytherapy known as proton radiation. The proton beams are aimed precisely at the cancer. This type of radiation reduces the risk of damage to the surrounding healthy eye tissue.
We have more information about radiotherapy to the eye area. It’s aimed at adults with eye cancer.
Surgery
Your child might need surgery to remove the eye (enucleation) if 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 which 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.
Chemotherapy uses drugs to kill cancer cells. Your child might have their chemotherapy in one or more of the following ways:
into their bloodstream (intravenous) 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 (vitreous)
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.
Your child’s doctor might suggest chemotherapy 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. Your child might have between 4 to 6 cycles. Each cycle lasts between 3 to 4 weeks.
Other chemotherapy drugs used for systemic chemotherapy include:
cyclophosphamide
ifosfamide
doxorubicin
topotecan
If your child is having chemotherapy into a blood vessel or as an injection directly into the jelly part of the eye, they commonly have one of the following:
melphalan
carboplatin
topotecan
Your child usually has chemotherapy into the blood vessel every 3 weeks. They have between 2 to 4 cycles. If they are having it into the jelly part of the eye, your child has between 2 to 6 cycles. The injection is every 7 to 14 days.
Side effects
The side effects of having chemotherapy into the artery or vitreous of the eye can be different to those intravenous chemotherapy. These might include side effects such as:
inflammation
droopy eyelid
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.
Find out more about chemotherapy drugs and possible side effects
Going to sleep (general anaesthetic)
Your child will receive most of their treatment, tests or scan while they are asleep, unless they are 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 and your child breathes in the anaesthetic gas until they fall asleep. A member of the team lets 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 will take your mobile number to 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, either within a mainstream school or in 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 such as carboplatin and cisplatin can cause changes to your child’s hearing. 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 them to develop a second cancer many years into the future. If your child has an inherited retinoblastoma second cancers can occur in later life. Your child’s doctor will talk this through with you and answer any questions you may have about it.
Trilateral retinoblastoma is very rare type of cancer and children with inherited retinoblastoma can develop this. It starts in the centre of the brain, from the pineal gland . The pineal gland is just behind the brain stem. It makes the hormone melatonin that controls sleep. Symptoms include headaches and feeling and being sick.
Your doctor will talk to you about this if your child is at risk. They will monitor your child very closely.
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 and 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 for a close look at their eyes.
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 affected.
As well as the doctors and nurses on the ward, you and your child will be supported by a number of professionals during and after treatment. These include your GP, cancer nurse specialists, social workers, play specialists and a psychologist or psychotherapist.
Talking to other people who are experiencing the same thing can also help. Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.
The Childhood Eye Cancer Trust is a charity for families and people affected by retinoblastoma. It offers support and information, funds research and raises public awareness of this rare cancer.
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