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 develop in both eyes. Children with both eyes affected often have a parent who has retinoblastoma. Every child of an affected parent is usually screened regularly from birth.

    Signs & Symptoms

    Retinoblastoma is usually noticed as a white appearance of the pupil, which tends to reflect light like a cat's eye. A squint 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 CT scan may also be done and usually lumbar puncture will be done to see if there is spread of the tumour. (Refer to sheet on tests and procedures)

    Treatment

    The number, site and size of tumours determines the type of treatment. A combination of chemotherapy and radiotherapy may be used to treat retinoblastoma. Sometimes, when the tumour is very large and the vision in the eye has been lost, it is 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.