Retinoblastoma Retinoblastoma is the most common malignant intraocular tumor in neonates, occurring in in 20,000 infants It can be unilateral or bilateral and can be inherited or sporadic in nature Retinoblastoma usually presents by 13 to 18 months if not captured earlier on routine examination (50% of cases are diagnosed by finding an abnormality of red reflex during routine examination or by a parent) Mutation or inactivation of the tumor suppressor gene is thought to contribute to its development Children of affected parents with the inherited or bilateral form have a 45% chance of developing this tumor As the tumor progresses anteriorly, strabismus due to deterioration of vision occurs Fundal examination reveals posterior chamber mass with calcification The globe starts to enlarge because of the large mass or secondary glaucoma Secondary glaucoma also causes photophobia Other manifestations include pseudohypopyon and spontaneous intraocular bleeding CT scan or MRI is necessary for determining the degree of extraocular spread and confirming the diagnosis Early detection and treatment is of utmost importance because 5-year survival is approximately 98% if the tumor is confined to the globe