What is retinoblastoma?
Retinoblastoma is a type of eye cancer that occurs in the retina of one, or both, eyes. The eyeball itself has a lens at the front (under the iris) which focuses the light coming into the eye onto the retina – the light-sensitive lining at the back of the eye. The retina then sends a message along the optic nerve allowing us to see. Treatment will aim to remove the tumour and if possible, save the sight in the eye.

Who does it affect?
Children who are diagnosed with retinoblastoma are generally under five years old. It is estimated that 40% of cases are inherited because of a certain gene passed down from parent to child. Children at risk are screened regularly until they are five.

What are the symptoms?
The most common symptoms are a strange-looking pupil that does not reflect the light, a squint or a red, sore eyeball.

How is it diagnosed?
Retinoblastoma is diagnosed by its appearance. The eye will be examined whilst the child is under general anaesthetic. Other tests, such as an ultrasound and lumbar puncture will also be used to see if the tumour has spread into any surrounding tissue.

How is it treated?
The treatment will depend upon the stage of the cancer – in other words its size, and if it has spread. If the tumour is small and has not spread then the treatment is often a local therapy involving freezing the tumour (cryotherapy) or laser therapy where a laser removes the tumour. Larger tumours are treated with chemotherapy, radiotherapy and sometimes surgery. Chemotherapy is given to try and shrink the tumour. It can be used before the local therapy treatments are used, or when there is a risk of the cancer spreading. Radiotherapy will aim to destroy the tumour using targeted high-energy rays. This can be effective when other treatments have not worked. Sometimes, if a tumour is large the eye itself may need to be removed. In these cases an artificial eye will be fitted but sight in that eye will be lost. 

Side-effects
The side-effects associated with chemotherapy are tiredness, sickness and diarrhoea, temporary hair loss and infection, all of which can be managed with help from the child’s Consultant or nursing team.

Long term side-effects
Some children may go on to develop longer term side effects. Parents can discuss these with their child’s Consultant.

Follow-up care
All children will need to be monitored regularly at an outpatient's clinic. The Consultant will be looking for any signs that the cancer has returned (recurrent cancer) and checking the functions of major organs to make sure that they have not been affected by the cancer treatment. 

Source: www.clicsargent.org.uk