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.
Retinoblastoma is often curable when it is diagnosed early, but if it is not treated promptly, this cancer can spread beyond the eye to other parts of the body. When tumor is exclusively localize into the eye, the majority of patients can be cured by therapeutic treatments including enucleation or conservative treatments using, intravenous and/or intraarterial chemotherapy, cryotherapy, local thermotherapy or brachytherapy. External beam radiotherapy is increasingly restricted due to the risk of late effects. The potential risks of late effects associated with chemotherapy and carboplatin include increased risk of second cancer, as well as short-term important side effects. Besides, due to the lack of blood supply to vitreous seeding, chemotherapy drugs could not reach the vitreous body through blood-retinal barrier. This is one of the important limiting factors which triggered intravenous chemotherapy as a new injection technique for vitreous disease in retinoblastoma.
Last date updated on July, 2014