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It occurs mostly in children younger than 5 years and accounts for about 3% of the cancers occurring in children younger than 15 years. Adult cases have also been clinically recorded. The estimated annual incidence is approximately 4 per million children. It begins with white blotches in one or both eyes (leukocoria) which can be seen in photographs (this is distinct from the red-eye effect which is normal); or when light reflects off the eye, as when watching television.
The tumor may begin in one or both eyes. Retinoblastoma is usually confined to the eye but can spread to the brain via the optic nerve.
As the retina is the light-sensitive part of the eye necessary for vision, loss of vision occurs.
Until recently the only treatment was to remove the affected eyeball before the cancer spread. Chemotherapy is the treatment of choice for most unilateral cases. However with locally advanced disease external beam radiation may be needed and if both eyes are involved enucleation may be the only option. Affected children in developing countries present with advanced features and usually die of metastatic spread. In its initial stages, retinoblastoma is very similar to Coats disease, a non-cancerous retina disease. Coats' Disease should be ruled out before enucleation is done. A mis-diagnosis of Retinoblastoma accounts for the greatest number of Coats' disease eyes being enucleated.
Many children with bilateral retinoblastoma can be treated with a preservation attempt. Tumor chemoreduction with carboplatin and other drugs may reduce the tumor volume making them amenable to local therapies 
Local therapies include-
Laser therapy (Uses infrared laser light to precisely destroy the blood vessels surrounding a tumor.)
It is important that children with retinoblastoma are treated in specialist centers.
Brachytherapy with beta-emitting eye applicators have also been a successful major treatment. BEBIG (GmbH-Berlin-Germany) produces various kinds of ruthenium ophthalmic applicators for treating retinoblastoma.
In October 2007, researchers have identified the specific cell that causes retinoblastoma.
The condition reached the headlines in May 2007, when Utah Jazz point guard Derek Fisher revealed that his 10-month-old daughter had the condition. Fisher missed Game 1 and half of Game 2 of the playoff series versus the Golden State Warriors to be with his daughter for her surgery in New York City.
See Ben Underwood for a case who compensated for the resulting blindness by developing human echolocation.
After losing his vision due to a Retinoblastoma operation, Eddie Timanus became a sportswriter for USA Today and tied a record by winning five consecutive episodes of Jeopardy!, at which point he was forced to "retire" from Jeopardy! competition.
|This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Retinoblastoma". A list of authors is available in Wikipedia.|