NCCN Christchurch Retinoblastoma Overview Incidence 3 of childhood cancers in West 15 in LMIC 60 70 isolated unilateral 30 40 hereditary Bilateral RB1 mutation autosomal dominant ID: 935217
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Slide1
Retinoblastoma
Rob Corbett
NCCN
Christchurch
Slide2Retinoblastoma
Overview
Incidence
3% of childhood cancers in West15% in LMIC60 – 70% isolated, unilateral30 – 40% hereditary:Bilateral RB1 mutation – autosomal dominantSensitive to chemotherapy and RTCure:>95% in DCsJust >50% in LMICs
Presentation
Early:
Leukocoria
Squint
Later:
Exophthalmos
Painful eye
Regional LNs
CNS signs
Weight loss
>50% symptoms/signs for > 6 months
Slide3Retinoblastoma
Slide4Retinoblastoma
Assessment/Investigations
Ophthalmologist – typical appearance
Careful clinical examRadiologyCTMRIBlood countCSFBone marrows
Slide5Retinoblastoma
Treatment
Stage I 6 courses carboplatin, etoposide, vincristine (CEV) very curable
Stage II CEV x 6 + RT > 50% curableStage III CEV x 2 surgery CEV x 4 + RT ~50% curableStage IV palliation NB is early diagnosisNB is
treatment refusal