Raising awareness: Retinoblastoma
Retinoblastoma is a rare form of cancer that develops in the immature cells of a retina in the eye.
The retina is a thin layer of tissue that lines the back of the inside of the eye. Located near the optic nerve, its purpose is to receive light focused from the lens and convert it to neural signals that the brain subsequently translates for visual recognition. (1)
In the UK alone around 40 children are diagnosed with retinoblastoma annually. The majority of those affected are under five years of age, with more than 40 per cent of cases occurring within the first year of life.(2)
Retinoblastoma can affect one eye which is referred to as unilateral. This is the case for 2 out of 3 children diagnosed. When it occurs in both eyes it is referred to as bilateral. If it affects one eye, it tends to be diagnosed later on. It is important to note that all children with bilateral tumors have the inheritable form of the disease, in comparison to only 10% of unilateral cases that have the inheritable form.(2)
Signs and symptoms of retinoblastoma
The signs and symptoms of retinoblastoma include but are not limited to:
- A white reflection on the pupil: this presents in photos, where only the healthy eye appears red from the flash, and the other has a white appearance.
- A change in the colour of the iris. This is commonly occurs in one eye however can occur in a specific area of the eye
- Decrease in vision. The effected child will be unable to focus on objects and/or unable to control their eye movements
- A red and/or inflamed eye. This usaully presents without pain (3)
In the case of family history, children are screened for retinoblastoma before symptoms are detected. Follow ups will usually continue until the age of five. Where there is no family history, diagnosis is usually made by conducting the red reflex test (initially performed by a general practitioner). Whilst a normal eye will reflect red, if a white reflection is seen, it may be a sign of numerous eye conditions including cataracts and retinal detachment. Such cases are urgently referred to a specialist ophthalmologist for further investigation.(3)
Steady medical advances have resulted in earlier diagnosis and treatment of retinoblastom. This has subsequently increased the success of treatment and survival rates. Late diagnosis is usually associated with poor prognosis. Data collected between 2006 and 2010 showed that the five year survival rate stood at 100%. It is important to note however that survival may sometimes be at the expense of some or all of the child’s eyesight.(2)
If your or any one you know has been effected by Retinoblastoma, the following organisations have been set up to provide you with help and support. These include but are not limited to;
- Childhood Eye Cancer Trust (CHECT) actively provide sufferers and others effected by the disease with information about retinoblastoma in terms they can easily understand. Their contact details are as follows: 020 7377 5578 (Phone lines operating Monday to Friday, 9am to 5pm).
- Cancer Research UK Have set up a helpline where you can speak to a specialist cancer nurse who will be able to provide you with information and support. Their number is 0808 800 4040 (Operating between Monday to Friday, 9am to 5pm).
- Macmillan Have a helpline that offers specialist support and information – 0808 808 00 00 (perating Monday to Friday, 9am to 8pm) (3)