Retinoblastoma

Retinoblastoma (Rb) is a rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common malignant cancer of the eye in children, and it is almost exclusively found in young children.

Though most children survive this cancer they may lose their vision in the affected eye(s) or need to have the eye removed.

Almost half of children with retinoblastoma have a hereditary genetic defect associated with retinoblastoma, in other cases, it is caused by a congenital mutation in the chromosome 13 gene.

Classification

There are two forms of the disease, a heritable form and non-heritable form (all cancers are considered genetic in that mutations of the genome are required for their development, but this does not imply that they are heritable, or transmitted to offspring). Approximately 55% of children with retinoblastoma have the non-heritable form. If there is no history of the disease within the family, the disease is labeled "sporadic", but this does not necessarily indicate that it is the non-heritable form. Bilateral retinoblastomas are commonly heritable, while unilateral retinoblastomas are commonly non-heritable.