2 out of 5 cases of retinoblastoma are caused by a genetic abnormality, which is sometimes inherited from a parent. As such, children from families with a history of the disease are often screened shortly after birth and offered blood tests for to assess their risk of a tumour developing. Further screening is recommended every few months for 5 years.
Although this form of retinoblastoma is very well understood, it is not known what causes non-heritable retinoblastoma.
Sometimes, the cells in the eyes that mature to form the retina (retinoblasts) do not stop reproducing and can form a tumour on the retina.
Retinoblastoma most often occurs in children 5 years and younger, with around 80 cases diagnosed each year in the UK.
A white that does not reflect light, called leucocoria, may mean that a retinal tumour is present. This could be spotted when a picture of your child is taken using flash photography. The back of the eye will normally reflect red. Some children may have a squint or red, painful eye(s)
An eye examination and imaging test, given by an optician or Consultant Ophthalmologist, can diagnose retinoblastoma. Tests are usually done while the child is asleep under anaesthesia. A biopsy is not usually necessary since retinoblastoma can be diagnosed by their appearance.
Further tests may be conducted to determine the position, size and stage of the retinoblastoma. Tests include ultrasound scan, MRI scan, lumbar puncture, bone marrow test, or blood test.
Stages Of Retinoblastoma
- Intraocular retinoblastoma – the earliest stage of retinoblastoma can be found in one or both eyes, but has not yet spread outside the eye.
- Extraocular retinoblastoma – this type of cancer has either spread outside the eye to other parts of the body.
- Recurrent retinoblastoma – the cancer has returned or spread in the eye or to other parts of the body after being treated.
Retinoblastoma is highly curable since it is usually found before it spreads outside of the sclera (white of the eye).
The type of treatment used depends on the stage of the cancer and the position, size and number of tumours present.
For smaller tumours, treatment is given to the eye itself, while your child is asleep under anaesthetic. Treatments include:
- Thermotherapy – heat is used to destroy cancer cells, via a laser directed at the tumour. Thermotherapy may be combined with chemotherapy or radiotherapy, as heat can improve the effectiveness of these treatments.
- Laser therapy – a laser is used to heat the tumour. A number of sessions may be necessary, at intervals of 3-4 weeks.
- Cryotherapy – tumour cells are destroyed by means of being frozen. More than one session may be necessary, done at monthly intervals.
- Plaque – a small radioactive disc is stitched over the tumour on the outside of the eye. The disc needs to stay in place for up to four days while the radiation destroys the cancer cells. This is done for slightly larger tumours, or tumours that have not been successfully treated with other methods.
- Chemotherapy – cytotoxic anti-cancer drugs are used to destroy cancer cells. Chemotherapy may be given before the local treatments mentioned above, to help shrink the tumour and make treatment more successful. Chemotherapy can also be used if the cancer has spread to other parts of the body, or if there’s a risk that it may spread.
- Intra-arterial chemotherapy – a new approach in some cases is to give chemotherapy directly into the blood vessel that supplies the affected eye. It is not commonly used and tends to be reserved for tumours that haven’t responded well to the standard treatment or if a tumour comes back.
- Surgery – if the tumour is very large and the vision in the eye is lost, your child is likely to need an operation to remove the eye. The specialist will only do this if it is absolutely necessary. An artificial eye is then fitted.
- Radiotherapy – radiotherapy treats cancer by using high energy rays from a machine to destroy the cancer cells, while doing as little harm as possible to normal cells. It can be given to the whole eye but does have some effect on the surrounding tissue. Radiotherapy for retinoblastoma is normally only used when other treatments have not worked well.
Our Appointments Team have a dedicated and caring approach to finding you the earliest appointment possible with the best specialist.
If you do not have a GP, then we have an in-house private GP practice that you can use. Alternatively we can suggest the most appropriate course of action for you to take, given your location and individual circumstance.
If you have medical insurance (e.g. Bupa, Axa PPP, Norwich Union), you will need to contact your insurer to get authorisation for any treatment and, in most cases, you will require a referral letter from your GP.