The success of primary chemotherapy for group D heritable retinoblastoma.
OBJECTIVE
To report the ocular survival and event-free survival following primary multiagent chemotherapy for group D, heritable bilateral retinoblastoma (RB).


METHODS
The RB database was used to identify children with heritable, bilateral RB treated with primary chemotherapy (six cycles of vincristine, etoposide and carboplatin). Only Group D eyes with more than 12 months' follow-up were analysed. The timing, number and type of salvage treatments were recorded. Kaplan-Meier estimates for the ocular survival and event-free survival (percentage of eyes that avoided external beam radiotherapy and/or enucleation) were performed as a function of time.


RESULTS
Of 18 group D eyes, two (11%) were treated successfully with chemotherapy alone, nine (50%) underwent successful salvage treatment, and seven (39%) were enucleated. The median time from completing chemotherapy to enucleation was 9 months (range 4 to 25 months). Ocular survival was 67% at 2 years. External beam radiotherapy proved successful salvage treatment in five of nine eyes, so the event-free survival was 34% at 2 years.


CONCLUSIONS
Multiagent chemotherapy alone is rarely sufficient for the preservation of group D eyes. External beam radiotherapy and plaque radiotherapy remain important salvage treatments for advanced, heritable retinoblastoma.
Cohen
V M L
VM
St Bartholomew's and the Royal London Hospital, West Smithfield, London EC1A 7BE, UK. victoria.lendrum@gmail.com
Kingston
J
J
Hungerford
J L
JL
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D016190:Carboplatin / Q000008:administration & dosage; D002675:Child, Preschool; D018572:Disease-Free Survival; D005047:Etoposide / Q000008:administration & dosage; D015353:Eye Enucleation; D005260:Female; D018095:Germ-Line Mutation / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control; D019572:Retinal Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000601:surgery; D012175:Retinoblastoma / Q000188:drug therapy* / Q000235:genetics / Q000601:surgery; D016160:Retinoblastoma Protein / Q000235:genetics; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D016160:Retinoblastoma Protein; D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin