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Retinoblastoma
Retina & Vitreous
Key Points
- •Retinoblastoma is the most common intraocular malignancy of childhood - caused by biallelic RB1 inactivation (chromosome 13q14); follows Knudson's two-hit hypothesis
- •Leukocoria is the most common presenting sign (~60%); calcification on CT/US in ~95% is the key diagnostic feature; NEVER biopsy
- •ICRB classification (Groups A–E) guides management: focal therapy (A–B), chemoreduction + focal (C–D), enucleation (E); IAC and intravitreal melphalan have improved globe salvage
- •Heritable RB (~40%): bilateral, multifocal, risk of second primaries (osteosarcoma) and trilateral RB (pineoblastoma); survival >95% in developed countries
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17 more sections are included with the Clinical plan.
- Definition
- Epidemiology
- Aetiology and causes
- Pathogenesis
- Classification
- Risk factors and associations