Retinoblastoma

General: Malignant tumor arising in one or both retinas of 727f58h young children, usually under the age of 2 years; usually unilateral; autosomal dominant; most common intraocular malignancy of childhood; incidence is one in 20,000 live births; origin is questionably neuroectodermal cells capable of multipotentiality; one third of patients have heritable (bilateral or have a positive family history) autosomal dominant and two thirds are sporadic; genetic transmission obeys two-mutation hypothesis of Knudson; trilateral retinoblastoma is bilateral retinoblastoma plus midline central nervous system tumor (most commonly pinealoma); most common second tumor is an osteogenic sarcoma (begins in second decade).

Ocular: Hyphema; hypopyon; corneal tumor; lid edema; endophthalmitis; exophthalmos; intraocular calcification of globe; heterochromia; neovascularization of iris or retina; papilledema; panophthalmitis; retinoblastoma extension into orbit and choroid; cat's-eye reflex; leukocoria; mydriasis; vitreous hemorrhage tumor seeding; esotropia; exotropia; glaucoma; visual loss.

Clinical: Metastasis into the lymph system, bone marrow, and subarachnoid space; basal meningitis; death.

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Ohira A, et al. Glial-neuronal-and photoreceptor-specific cell markers in rosettes of retinoblastoma and retinal dysplasia. Curr Eye Res 1994; 13:799-804.