Key Statistics for Retinoblastoma

Learn about key statistics for retinoblastoma, including how common it is, who is most affected, and the outlook for those with a diagnosis.

How common is retinoblastoma?

Retinoblastoma is a rare cancer of the eye. It is the most common tumor affecting the eye in children, and accounts for about 3% of all childhood cancers. About 300 –350 cases are diagnosed in the United States each year.

Who is most affected by retinoblastoma?

2 out of 3 retinoblastoma tumors are diagnosed in children younger than 2 years of age, and almost all retinoblastoma is diagnosed in children younger than 5.

Changes in the RB1 gene, which normally controls cell growth, are found in most retinoblastomas. In some children, changes in the RB1 gene are present in cells throughout the body, not just in the tumor. When this happens, it is called heritable retinoblastoma. 4 out of 10 children diagnosed with retinoblastoma have heritable retinoblastoma, which can put them at risk of other cancers and passing the gene to their own children in the future.

Retinoblastoma can affect one eye (unilateral) or both eyes (bilateral), and in rare cases be associated with a tumor in the brain (trilateral). About 1 in 3 children with retinoblastoma have bilateral retinoblastoma. Bilateral disease tends to occur in younger children compared to unilateral disease. It is also more likely in children with heritable retinoblastoma.

Trilateral retinoblastoma is a rare syndrome in children with heritable retinoblastoma. About 5% of children with heritable retinoblastoma develop midline tumors in the brain.

What is the outlook for those with retinoblastoma?

Overall, more than 9 out of 10 children in the US with retinoblastoma are cured. Retinoblastoma that spreads outside of the eye can be harder to treat.

Visit the American Cancer Society’s Cancer Statistics Center for more key cancer statistics.