Abstract
Retinoblastoma (Rb) is a common tumor of childhood. Approximately half of the patients with Rb carry a somatic mutation of a retinoblastoma (Rb1) gene, this form of Rb is called hereditary; usually both eyes are affected.
A second form of Rb is nonhereditary, unilateral Rb. The diagnosis of Rb is made by ophthalmologists during a detailed examination under general anesthesia that determines a stage of retinoblastoma, number of tumors in an affected eye and describes a seeding.
Pediatric oncologists add other staging tests, including magnetic resonance imaging of a brain and orbits. Treatment of Rb includes local techniques performed by ophthalmologists and administration of systemic chemotherapy (or super-selective chemotherapy administered directly to a ophthalmic artery via catheterization technique), brachytherapy or external beam radiotherapy.
Overall survival of Rb is excellent in developed countries, but the significant percentage of the affected eyes has to be enucleated for an extensive Rb at diagnosis, or for persistence or recurrence of the tumor in the eyeball.