Aim: 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, 2 (11%) were treated successfully with chemotherapy alone, 9 (50%) underwent successful salvage treatment and 7 (39%) were enucleated. The median time from completing chemotherapy to enucleation was 9 months (range 4–25 months.) Ocular survival was 67% at 2 years. External beam radiotherapy proved successful salvage treatment in 5 of 9 eyes, therefore, the event free survival was 34% at 2 years.
Conclusion: 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.