Retinoblastoma Dr Trisha Macnair An abnormal pupil is a common sign of this cancer of the eye.

In this article

What is retinoblastoma? Symptoms

Causes and risk factors Treatment and recovery

Advice and support

Retinoblastoma is a malignant tumour that develops at the back of the eye. It originates in the cells of the retina, the light-sensitive part of the eye.

The most common signs of retinoblastoma are an abnormal pupil that tends to reflect light rather like a cat's eye, and a squint. The child's vision may also start to deteriorate and the eye may become red, inflamed and sometimes painful.

In two-thirds of cases the cancer is unilateral (affecting one eye). In the rest it's bilateral.

In 95 per cent of children, the tumour develops before the age of five. Occasionally, babies are born with a retinoblastoma. There's an increased risk of developing other tumours later in life.

Every year in the UK, 40 to 50 children develop retinoblastoma. Around 40 per cent of cases are caused by an inherited genetic problem - a faulty gene on chromosome 13.

Treatment for retinoblastoma has one of the highest success rates of all childhood cancers - nine out of ten children can be cured. Cryotherapy (a freezing treatment), radiotherapy, chemotherapy and surgery are used.

Antenatal and postnatal genetic screening can be offered, but testing for the retinoblastoma gene isn't easy because it's large and many different abnormalities may occur.

The test is only offered to those with certain types of retinoblastoma and where babies are known to be at high risk.

Childhood Eye Cancer Trust
Tel: 020 7377 5578
Email: info@chect.org.uk
Website: www.chect.org.uk

This article was last medically reviewed by Dr Orlena Kerek in March 2009