Retinoblastoma is a childhood cancer that forms in the retina. It is a progressive genetic disorder. The retina is the light sensitive part of the eye. The retina covers approximately 65% of the interior of the eye. In immature retinal cells, the cancer will develop in children from the time the eyes develop in the womb to 5 years of age. The tumor starts in the retina, the light sensitive layer of the eye, which allows the eye to actually see. “ It can occur in either one eye (unilateral) or two eyes (bilateral)”(Hulett, WashBurn, and Orenic). If the cancer is caught early, it is curable, but if caught late, it is deadly. That is why in developing countries, 87% of children worldwide with this genetic disorder will die. For developed countries, 97 % survive but will have moderate to severe visual impairment. Before enucleation was known to help retinoblastoma, it was incurable. It was considered incurable and death always occurred because inheritance patterns had not been established, leading back to more deaths. But in all actuality, the survival rate will drop with every decade of life when the patients have the genomic mutation. The genomic mutation is a gene mutation within every cell of the individual’s body. Those patients with that type of cancer have either bilateral disease or unilateral-multifocal disease. Those individuals have a predisposition for developing second cancers later in life. Retinoblastoma accounts for 3 % of cancer in children under the age of 15. (Cotton, Gartman, Ryan, 2)
Retinoblastoma would fit into the category of a single gene defect. It is very sporadic and spontaneous. Most patients do no receive a gene defect from their parents. But 50% of survivors will pass on the gene to their children. Although it is rare, it is possible for it to spread from the to the brain, the
Bibliography: Bolling, James, and Thomas Carollo. "Retinoblastoma." N.p., Sept. 2000. Web. 7 Mar. 2013. Cotton, Mario, Kristin Gartman, and Kristen Ryan. "Retinoblastoma." Stephen F., n.d. Web. 8 Mar. 2013. Hulett, Linda, Linda Washburn, and Jennifer Orenic. "Retinoblastoma." N.p., July 2007. Web. 8 Mar. 2013. "Problems of Retinoblastoma." British Medical Journal (1972): 67. Web. 9 Mar. 2013. Shields, Carol L., and Jerry A. Shields. "Diagnosis and Management of Retinoblastoma." Cancer Control Sept. 2004: 317-22. Web. 8 Mar. 2013. Wong, Lennie, and John Boice. "Cancer Incidence after Retinoblastoma." JAMA Network 278.15 (1997): n. pag. Web. 9 Mar. 2013.