Intraocular and extraocular retinoblastoma.

Retinoblastoma, the most common primary ocular malignancy of childhood, is a tumor in which the pediatrician and pediatric oncologist can now play a much more significant role in therapy. Developments in molecular biology have now made carrier testing and prenatal diagnosis feasible. In the near future, these developments should greatly augment the pediatrician's and pediatric oncologist's ability to offer accurate and appropriate genetic counseling for affected families. A practical staging system for extraocular retinoblastoma together with stage-related effective chemotherapy and radiation therapy was presented in this chapter. These modalities now make possible long-term survival for the majority of the 1 out of 8 children with retinoblastoma who would otherwise die from metastatic disease. Finally, 40 per cent of all children with retinoblastoma (those with the germinal mutation) are at lifelong risk for second, nonocular malignancies. The recognition that more than half of these children will actually develop second tumors by the fourth decade of life makes vigilant follow-up care for these patients a necessity.