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Children's Cancer Centre

Retinoblastoma

  • What is retinoblastoma?

    Retinoblastoma is a tumour of early childhood arising in the cells of the light sensitive lining of the eye known as the retina. In about two-thirds of children only one eye is affected but in one-third, tumours may develop in both eyes. Retinoblastoma is caused by a gene that controls the growth of cells in the eye. This gene change may be inherited from a parent who also had retinoblastoma. Every child of an affected parent should be screened regularly from birth, however most children with retinoblastoma do not have any other family history of this disease.

    Signs and symptoms

    Retinoblastoma is usually noticed as a white appearance or glow of the pupil, which tends to reflect light, similar to a cat's eye. Other times, a squint or a crossed, or turned eye may develop. Children with very large tumours may have a painful, red eye or poor vision.

    Tests

    The diagnosis is made by an ophthalmologist (an eye specialist) who examines the eye usually under a general anaesthetic. An ultrasound and MRI scan may also be done. Sometimes a lumbar puncture will be undertaken to see if there is any spread of the tumour outside of the eye (refer to sheet on tests and procedures).

    Treatment

    The number, size and site within the eye or eyes determines the best treatment. Chemotherapy and focal therapy (heating or freezing) may be used together or on their own treat retinoblastoma. Radiotherapy is used less often, but may be essential. When the tumour is very large or cannot be controlled, or the vision in the eye has been lost, it may be necessary to remove the eye.

    Follow up care

    After treatment has finished the child will be seen at regular intervals and monitored with examination of the affected eye/eyes under a general anaesthetic. Although many children may have near normal vision (depending on the treatment required) some will have partial loss of sight. Radiotherapy may cause some side effects. After the first few years the focus of follow up changes to monitoring growth and development and other possible later side effects of treatment.