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RETINOBLASTOMA

• Primary malignant neoplasm of the


retina that arises from immature retinal
cells
• The most common malignant eye tumor
of childhood.
• Responsible for approximately 1% of all
deaths from carcinoma in the age group
< 7 yrs of age
EPIDEMIOLOGY
• Retinoblastoma(Rb) is the most common intraocular malignancy in
children, and affects 1 in 15,000 to 1 in 18,000 live births.
• It represents almost 4% of all pediatric malignancies.
• Approximately 250-300 children are newly diagnosed with
retinoblastoma each year in the United States
• Higher rates occur in developing countries.
• M=F
Occurs In Familial And Sporadic Forms
 Familial
Bilateral – 40%
Sporadic
Unilateral-60%
GENETICS
• Loss or inactivation of both normal alleles of the
retinoblastoma gene
• DNA sequence localized to a small segment of
the long arm (the q14 region) of chromosome 13

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