About retinoblastoma
What is retinoblastoma?
Retinoblastoma is a cancer of the eye. It is a malignant tumour, which develops in the cells of the retina– the light sensitive lining of the eye. About two thirds of children with retinoblastoma have only one eye affected (unilateral retinoblastoma), and one third have both eyes affected (bilateral retinoblastoma). In very young children, who are diagnosed with retinoblastoma in one eye, it is possible for a tumour to develop in the second eye several weeks or even months afterwards. The number of tumours found in the eye also varies; sometimes only one tumour develops at the back of the eye, but in some circumstances there are several tumours, and these may require more than one type of treatment. Retinoblastoma has one of the best cure rates of all children’s cancers, with more than nine out of ten children being successfully treated.
How common is retinoblastoma?
Retinoblastoma is one of the less common childhood cancers and accounts for only about three per cent of all cancers under the age of 15 but accounts for ten per cent of cancer in infants. In the UK between 40 and 70 children develop retinoblastoma each year. The tumour usually develops before the age of five years and some children are born with retinoblastoma. Children with bilateral retinoblastoma tend to be diagnosed during the first year of life, while the peak age of diagnosis for children with unilateral retinoblastoma is two and a half years. Only a small number of cases are diagnosed after the age of five years.
What causes retinoblastoma?
Retinoblastoma occurs in two forms: a genetic, inheritable form, and a non-genetic, non-inheritable form. Approximately 40 per cent of children with retinoblastoma have the genetic form of the disease
Signs and symptoms of retinoblastoma
The two most common signs of retinoblastoma are, firstly leukocoria, an abnormal appearance of the pupil in the eye which tends to reflect light as a white reflex (like a cat’s eye), and, secondly, a strabismus (squint).
This image shows the abnormal appearance in this child’s left eye.
Other less common signs are that the white of the eye may become red, a darkening or change in colour of the iris, or an enlargement of the eye. If you notice any unusual signs in your child’s eye(s), which can sometimes be evident in photographs, please visit your GP or local optician for advice. For more advice for parents, please click here.
Diagnosis and investigation
The diagnosis of retinoblastoma is made by an eye specialist known as an ophthalmologist. The ophthalmologist will examine your child’s eyes under a general anaesthetic, while your child is unconscious. For more information about the investigation, please click here.
Treatment of retinoblastoma
There are a number of different methods of treatment available and the decision as to which is the most suitable will be made by your child’s ophthalmologist, who will take into account the size, location and number of tumours within the eye(s) and the also the potential to save your child’s vision.
