|Retinoblastoma is the most common intraocular malignant tumor in childhood with an incidence of 1 for 20,000 births which occurs during the first five years of life. The retinoblastoma cell of origin has been debated for over a century; however, we now have a detailed understanding of the neuronal pathways that are deregulated in the primitive cells of the retina. The role of genomic instability of the retinoblastoma gene Rb1 localized on chromosome 13q and the most relevant epidemiological issues concerning retinoblastoma have recently been pointed out to explain the variegated clinical expression of the disease. The most common signs of retinoblastoma are a visible whiteness in the pupil called leukocoria and a strabismus. The unilateral form of the disease represents 70% of cases; however 30% of children develop the bilateral form in both eyes.
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