Most common intraocular cancer of childhood with approximately 2000 cases diagnosed annually in India . The incidence of bilateral tumors is strongly age dependent with 42% of the occurring in children < 1 year of age .About one third of cases are bilateral. Bilateral and familial retinoblastomas are caused by a Germline mutation and are thus a heritable tumor. Approximately 40% of cases are Hereditary. The retinoblastoma gene is a tumor suppressor gene responsible for the Hereditary/Bilateral cases. Patients with Retinoblastoma most frequently come to medical attention because of the presence of Leucocoria ( White reflex ). Strabismus, conjuctival erythema and decreased visual acuity are other presenting complaints. In our country most cases can present as an orbital mass due to late presentation. |
