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01-01-2016 | Ophthalmologic cancers | Article

153. Retinoblastoma

Authors: MB, PhD, MRC Ophth, FRCS Mandeep S. Sagoo, MD Carol L. Shields, MD Jerry A. Shields

Publisher: Springer Berlin Heidelberg


Retinoblastoma (Rb) is a malignant tumour of immature progenitor retinal cells. Although Rb is the most frequently encountered intraocular malignancy of childhood, it is a rare disease, occurring in 1 in 14,000 to 1 in 20,000 live births depending on the population. In the United Kingdom, there are approximately 50 new cases per year, and in the United States, this figure is around 250–300. Untreated Rb is universally fatal, so early detection, diagnosis and referral to a specialist centre for appropriate management are essential for survival of the child. Misdiagnosis with an inflammatory or infective uveitis is unusual. The possibility of retinoblastoma should be considered in children with presumed uveitis that appears to be atypical.

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