RB Facts

What is it…

Retinoblastoma is the most common eye cancer in children. It is a life-threatening cancer of the retina within the back of the eye. Retinoblastoma is generally discovered in babies between the ages of 6 and 24 months, although it can be found at earlier or later ages. Retinoblastoma occurs in approximately 1 in 15,000 live births, and it is estimated to affect approximately 250 to 300 children each year in the United States. Worldwide, about 5,000 children develop retinoblastoma each year.

Who gets it…

Retinoblastoma occurs equally in boys and girls and it is seen in all races. Retinoblastoma affects only one eye in about 70% of patients and it affects both eyes in 30% of patients. In some instances, there is a family history of retinoblastoma in a parent or relative, but in the majority of cases, there is no other member of the family affected with the tumor.

What causes it…

The cause of retinoblastoma is unknown. It has not been related to nutrition, smoking, drinking, or any maternal problem during pregnancy. It is not related to environmental toxins. It can develop in an otherwise healthy child despite a normal birth and early development.

What does it look like…

Retinoblastoma develops from a single affected cell in the retina and it is invisible at its inception. Later, a small white nodule is noted in the retina, but this is not visible to the parents and it does not affect the child at this point. With time, the tumor grows into a larger white vascular tumor that causes the child to painlessly lose vision and this can manifest with a drifting eye or an eye with a white or glassy-appearing pupil. At this point, the tumor is visible to the parents or grandparents and the child is typically taken to the doctor.