The retina is the light-sensitive lining on the back of the eye. The lens at the front of the eyeball focuses the light coming into the eyes onto the retina. The retina then sends a message along the optic nerve allowing you to see.
Retinoblastoma is usually diagnosed in children under five years old, although it can affect people of any age. It is estimated that about 40% of cases are caused by an abnormal gene passed down from parent to child.
Children born into families with a history of this cancer will usually have regular checks for signs of retinoblastoma. This screening usually starts soon after birth and continues every few months for five years.
If your child is being screened for retinoblastoma, a developing tumour may be picked up before you notice any other symptoms. If this screening is not taking place, the first symptoms you may notice in your child include an unusual looking pupil that does not reflect the light, a white reflection in the pupil in flash photographs, a squint, or a red, sore eyeball.
The eye will be examined by an eye specialist while your child is under general anaesthetic. Retinoblastoma can usually be diagnosed just by its appearance, so it’s not normally necessary to take a tissue sample. Other tests, such as an ultrasound and lumbar puncture may also be used to see if the tumour has spread into any surrounding tissue.
If your child has been diagnosed with intraocular retinoblastoma, this means the cancer has not begun to spread to other parts of the eye or the surrounding tissue. Extraocular retinoblastoma means that the cancer has spread beyond the eye into the surrounding tissue or to other parts of the body.
The treatment your child receives will depend on the stage of their cancer. Your child may be referred to a specialist retinoblastoma centre who will oversee any local treatment or surgery. If the tumour is small and has not spread, then a local therapy will be used. This is a treatment that focuses on the eye itself. The tumour may be frozen using a process called cryotherapy or removed using a laser (this is known as laser therapy). Larger tumours are usually treated with chemotherapy or surgery.
Chemotherapy involves using anti-cancer drugs to target the cancer cells in the body. It can be used to shrink the tumour before local therapy takes place, or if there is a risk of the cancer spreading. Chemotherapy can usually be given at your local centre.
Sometimes, if a tumour is large, the eye itself may need to be removed. An artificial eye will be provided, and these often look incredibly realistic, but sight will be lost in that eye.
If other treatments have not been effective, your specialist may suggest using radiotherapy to target the tumour with high-energy rays. Alternatively, your child’s specialist may recommend that he or she has a specialised form of radiotherapy called proton beam therapy, which can include travelling abroad.
The side effects your child will experience depend on the treatment. If your child is undergoing chemotherapy, he or she may experience tiredness, sickness, diarrhoea and temporary hair loss. Your child may also be at greater risk of infections.
Radiotherapy can also make your child feel tired and sick. He or she might experience skin irritation similar to sunburn and hair loss in the area that is being treated.
Talk to your specialist or nurses about how you can manage these side effects, or see the side effects section of our website for more information.
After your child's treatment is complete they will still need to be seen regularly at an outpatient clinic. At this clinic, your child’s specialist will be looking for any signs that the cancer has returned (this is known as a relapse). They will also check that your child’s major organs, such as the heart and lungs, are still functioning correctly and haven’t been affected by the cancer treatment.
Once your child’s risk of relapse has reduced, ongoing follow-up will focus on looking out for potential long-term side effects of treatment. The risk of this depends on the type of cancer your child had and the treatment received. A personalised surveillance plan is usually created that outlines the specific long-term follow up and ongoing investigations that your child will require.
Many people are at low risk of future health problems but some will have significant ongoing health needs. Talk to your child’s specialist about the potential long-term side effects of their treatment.
The underlying genetic abnormality that caused the retinoblastoma may also increase the risk of your child developing other conditions. It’s important to seek prompt medical advice if you have any concerns.
- Read about the different treatments for cancer
- Prepare for life on the ward with our practical tips for hospital stays
- Parents tell us it's hard talking to their child about cancer and treatment. Here are our tips and tools on how to make it easier
Updated November 2017, next review due 2019.