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Concept
Retinoblastoma
Summary
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 primary malignant intraocular cancer in children, and it is almost exclusively found in young children.
Though most children in high income countries 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 13q14 (retinoblastoma protein).
Retinoblastoma is universally known as the most intrusive intraocular cancer among children. The chance of survival and preservation of the eye depends fully on the severity. Retinoblastoma is extremely rare as there are only about 200 to 300 cases every year in the United States. Looking at retinoblastoma globally, only 1 in about 15,000 children have this malignancy but these numbers continuously increase.
Intraocular malignancies are more curable rather than extraocular malignancies due to early diagnosis and an early treatment prognosis. During infant screenings, if they incorporate an eye screening like they do a hearing screening, it can be detected at an earlier age, therefore, preventing its spread. Leucocoria is the primary indication of retinoblastoma and is when the cancer is still intraocular, meaning inside the eye. When light is reflected by the white tumor, the view of the red retina is blocked. Retinoblastoma can be curable after the initial sign and up to six months, if the tumor is intraocular. If you do not visit an ophthalmologist with signs of leucocoria within a reasonable amount of time, the delay in the diagnosis could lead to a more severe prognosis. Due to a delay in the diagnosis, it could result in proptosis which is then considered extraocular, the most severe.
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