Retinoblastoma

Retinoblastoma (RB) is a rare form of cancer affecting the retinal photoreceptor cells. Although the disease is very rare, it is the most common ocular malignancy in children and the third most common cancer to affect children —- occurring in one out of every 15,000 births.

There are two types of retinoblastomas; one is hereditary and affects both eyes (occurs in 10% of cases) and the other type is non-hereditary and affects only one eye. Although the cancer is genetically determined, only 6 percent of newly diagnosed RB patients are found to have a positive family history of the disease. In all cases, genetic counseling is important for children with a germ-line mutation.

Treatment

Early detection of RB greatly enhances the possibility of a cure and the preservation of the greatest amount of vision. The treatment of RB depends on the size and location of the tumor and whether one or both eyes are involved. Treatment options include laser, chemotherapy, radiation, and removal of the eye. With earlier detection and improved treatments, the prognosis for vision and life for RB patients has improved significantly in the past twenty years. If left untreated, RB rapidly expands to fill the eye, extend along the optic nerve to the brain, and ultimately causes death.