Following enucleation, adjuvant chemotherapy is given to a small proportion of children who on histological grounds, are identified as being at risk of developing metastatic disease. For children with unilateral disease, there is a small chance that they may carry a new germ-line mutation or that one of the parents may be an 'unaffected' gene carrier. An essential part of the initial assessment of a child with retinoblastoma should include a careful examination of both parents' eyes for the presence of spontaneously regressed retinoblastoma. With the technology now available, it is becoming possible to identify those patients who carry germ-line mutations. This means that in the not too distant future, we shall be able to distinguish between the hereditary and the non-hereditary form of unilateral retinoblastoma as part of the routine assessment.
Late deaths are mostly due to second malignancies rather than to metastatic retinoblastoma.
The improvements in survival and quality of vision observed over the past decade have been achieved by the judicious use of surgery, radiotherapy, chemotherapy and focal methods of treatment. New techniques of radiotherapy have been introduced to reduce radiation damage to the normal structures of the eye. Effective chemotherapy is now available to prevent the spread of retinoblastoma outside the eye and intraocular disease can be cured by the use of chemotherapy in combination with either radiotherapy or thermotherapy. For children with a visual impairment, the development of new electronic devices is helping them achieve their full potential with regard to education, career prospects and lifestyle. The future Recognition of the retinoblastoma gene and identification of gene carriers means that ante-natal diagnosis can be offered to an increasing number of parents.