Retinoblastoma (reh-tin-oh-blast-oma) is a malignant cancer of early childhood that arises from immature retinal cells in one or both eyes. Retinoblastoma can start growing at any time before birth up until about 3 years of age. Occasionally, it is not detected until ages 7 or 8.
The tumor occurs in one of every 15,000 live births, and only 10 percent of cases have a prior family history. This relatively uncommon tumor of childhood accounts for about three percent of the cancers in children under the age of 15, and is curable if caught early. About 60 percent of affected children develops a tumor in only one eye.
Among infants and children, common signs of retinoblastoma include having a white "glow" or "glint" in the pupil of one or both eyes, the presence of a white pupil in a color photo, and crossed or misaligned eyes. Early detection is vital. Red reflex screening and dilation of the pupils is strongly recommended for all newborn babies.
Left untreated, retinoblastoma can be fatal or result in blindness. Although it is rare, retinoblastoma can spread or metastasize outside of the eye to the brain, the central nervous system (brain and spinal cord), and the bones. In these cases, chemotherapy is prescribed by a pediatric oncologist and is administered through the peripheral blood vessels or into the brain for months to years after initial diagnosis of metastatic disease. Chemotherapy also may be given as primary therapy for intraocular disease (diseases within the eyeball).
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