Incidence

1. Retinoblastoma is the most common intraocular malignancy of childhood, occurring at a rate of 1 in 20,000 live births.

2. The average annual incidence is 11.0 new cases per million under 5 years of age.

3. Approximately 200 new cases of retinoblastoma occur each year in the United States.

4. Retinoblastoma accounts for 11% of cancers developing in the first year of life, but for only 3% of all cancers diagnosed in children younger than 15 years of age.

5. The average age at diagnosis is 18 months.

6. Approximately 40% of cases are hereditary. The majority of these patients present with bilateral disease with an average of three tumors per eye and presents in the first year of life. Of these, only 15% have an established family history of retinoblastoma.

7. The remaining 60% of cases are nonhereditary, most often presenting with unilateral and unifocal disease in the second and third years of life.

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