Retinoblastoma

Retinoblastoma is the most common and important malignant eye tumor of childhood, and after malignant melanoma it is the most common primary intraocular malignancy of the eye. This tumor was established as an entity by the Scotsman James Wardrop (1782-1869) in his Observations on the Fungus Haematodes. Wardrop appreciated that the tumor originated in the retina, and he was the first to advocate early enucleation in its treatment (12). The tumor, although accounting for only approximately 1% of deaths from cancer under 15 years of age (85), has attracted wide interest from a variety of disciplines, including epidemiology, genetics, and molecular biology. The seminal observation that retinoblastoma arises in susceptible developing retinal cells when both alleles of the retinoblastoma gene (RB1) are mutated, and the isolation and cloning of RB1 gene (56,115,124), have established this tumor as a model of oncogenesis. The literature on the tumor and the causative gene has grown dramatically. These advances have been accompanied by a more complete understanding of the histogenesis, growth, and differentiation of the tumor (219), as well as a more complete elucidation of its molecular genetics (21). Improvement in the treatment of retinoblastoma has led to reports of 5-year survival rates of 90% and 98% in Europe and the United States, respectively (255,300).