Retinoblastoma

Retinoblastoma Retinoblastoma: Cancer of the Eyes Retinoblastoma is the most common intraocular malignancy of infancy and childhood. Before this century, retinoblastoma was a fatal disease. The introduction of the ophthalmoscope, general anesthesia, and surgical enucleation has improved the situation. Today, the survival rate exceeds 90% in most industrialized countries. Retinoblastoma represents the phenotypic expression of an abnormal or absent tumor suppressor known as the retinoblastoma gene (RB1). The RB1 gene is found on the thirteenth chromosome The tumor develops from the immature retina. There are both hereditary and non-hereditary forms of retinoblastoma. In the hereditary form, multiple tumors are found in both eyes, while in the non-hereditary forms of retinoblastoma, only one eye is affected by only one tumor. Prior to the knowledge of the RB1 gene, children with retinoblastoma were classified as having either sporadic or inherited retinoblastoma. Clinically and histologically, inherited and sporadic tumors are indistinguishable from one another. Markers for the inherited variety include bilateral involvement and multifocal primary tumors in one eye. The absence of multiple tumors, however, does not exclude the possibility of inherited retinoblastoma. Historically, the retinoblastoma trait seemed to be transmitted in an autosomal dominant pattern. On occasion, a family will demonstrate a skipped generation indicating retinoblastoma, tumors, tumor, survival, incidence, eyes, eye, children, been, age, years, therapy, therapies, one, inherited, florida, enucleation, cases, cancer, vision, under, study, standard, spirs, results, rb1, radiotherapy, primary, per, million, gene, found, faptp, external-beam, data