Retinoblastoma is a rare type of eye cancer that affects children younger than five. It is usually detected and treated early in the UK, which is why over 98% of children with retinoblastoma are successfully treated.

Babies with a family history of retinoblastoma will be screened for the cancer soon after birth. This is generally done at one of the two treatment centres in the UK: 

• Birmingham Children’s Hospital
• the Royal London Hospital

Your GP can refer you to one of these centres if you had retinoblastoma as a child and are expecting a baby. It is also important to let your midwife or obstetrician know about any family history of retinoblastoma so any relevant tests can be arranged around the birth.

About 40 to 50 children develop retinoblastoma each year in the UK.

The following information is for parents or carers of children who have just been diagnosed with retinoblastoma. It explains:

• what retinoblastoma is and why it develops
• the treatment and follow-up your child can expect
• warning signs to be aware of, in case retinoblastoma comes back

About retinoblastoma

Retinoblastoma is cancer of the retina. The retina is the light-sensitive lining at the back of the eye. Retinoblastoma causes the cells of the retina to grow rapidly and out of control.

In about 40% of cases, retinoblastoma is caused by a faulty gene, which can affect both eyes. This may be inherited from the child’s parents or may develop randomly when the child is growing in the womb.

In the remaining 60% of cases, there is no faulty gene and only one eye is affected.

To check for retinoblastoma, an eye specialist will examine the child’s eyes by shining a light into them using special equipment called an indirect ophthalmoscope. If retinoblastoma is diagnosed or if there is a family history of retinoblastoma, the child will be checked by screening every few weeks or months. The frequency of this screening reduces over time but continues for five years.

If retinoblastoma has affected both eyes, it is usually diagnosed in the first year of life. If only one eye is affected, it may not be diagnosed until the child is around 18-30 months old.

Treatment and follow-up for retinoblastoma

Most cases of retinoblastoma are found early and are successfully treated before the cancer spreads outside the eyeball. When the cancer is entirely within the eye, it is known as intraocular retinoblastoma.

Your GP will refer your child to a specialist eye hospital for further checks. If retinoblastoma is suspected or diagnosed, your child will be referred to one of two retinoblastoma treatment hospitals, where they will be seen by a doctor trained in treating retinoblastoma.

Birmingham Children’s Hospital and the Royal London Hospital have specialist retinoblastoma teams. Screening and some treatment will be carried out there. If a child needs chemotherapy it will usually be done at a local children’s cancer centre and overseen by the retinoblastoma team at one of the two main hospitals.

The Childhood Eye Cancer Trust website has information about all the treatments for retinoblastoma mentioned below, including their side effects.

Treating early-stage retinoblastoma

Treating a small tumour that is completely inside the eye usually involves one of the following treatments:

• laser treatment to the eye (photocoagulation)
• freezing the tumour (cryotherapy)
• applying heat to the tumour (thermotherapy)

Treating larger tumours

Larger tumours will be treated with one or a combination of the following treatments:

• Radioactive plaque – if the tumour is not too large, a small radioactive disc is stitched over the cancerous cells and left in place for a few days to destroy the tumour, before the disc is removed.
• Chemotherapy – usually six cycles of anti-cancer drugs are given to kill the cancer cells. Sometimes, these anti-cancer drugs are injected directly into the blood vessel supplying the eye.
• Surgery to remove the eye – this is necessary if the tumour is large and the eye can no longer see. An artificial eye will be fitted. You can find out more information about having an artificial eye fitted on the National Artificial Eye Service website.

Children who lose sight in one of their eyes are usually able to adapt very quickly to seeing out of their healthy eye, without it affecting their life.

Your child may be offered treatment as part of a clinical trial. Search for clinical trials for retinoblastoma.

If the cancer has spread beyond the eye (extraocular retinoblastoma), it has the potential to spread around the body and is more difficult to treat. However, this is unusual in the UK.

Follow-up

Retinoblastoma requires a long period of follow-up tests which are first performed at a specialist centre.

After a period of treatment and observation, the follow-up tests usually take place at a local eye department.

Warning signs of retinoblastoma

It’s important to be aware of the warning signs of retinoblastoma. The signs may include:

• Abnormal reflex in the pupil – usually, the affected pupil reflects white, like a cat’s eye (see the picture on this page). This may be spotted in photos, where only the healthy eye appears red from the flash, or it may be noticed in a dark room or a room lit by artificial light.
• A squint – one eye may turn in towards the nose, or out away from the nose.
• Poor vision – your child may not fix their gaze on faces or objects or they may not be able to control their eye movements. This is more common when both eyes are affected.

Sometimes, the iris (the coloured part of the eye) may change colour, or the child may complain that they cannot see as well as they used to.

These signs may be due to something other than retinoblastoma, but you should still get them checked by a GP immediately. The GP should carry out a red reflex assessment.

The red reflex test involves using an instrument called a direct ophthalmoscope, which magnifies images and has a light at one end. When light is shone into your baby’s eyes, a red reflection should be seen as it is reflected back. If a white reflection is seen, it could be a sign of cataracts, retinoblastoma or a similar condition. In this case, your baby will be urgently referred to a specialist.

It is unusual for retinoblastoma to progress unnoticed until the child is older than five. In these rare cases, the eye may be red, sore or swollen, and there may be loss of vision in the affected eye.

More information and support

The Childhood Eye Cancer Trust (CHECT) provides information about retinoblastoma. It also offers help and support to parents and carers of affected children as well as adults who were affected as a child. CHECT can also put you in touch with other parents of affected children.