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The success of primary chemotherapy for group D heritable retinoblastoma
  1. Victoria M L Cohen (victoria.lendrum{at}gmail.com),
  2. Judith E Kingston (judith.kingston{at}bartsandthelondon.nhs.uk),
  3. John L Hungerford (john.hungerford{at}bartsandthelondon.nhs.uk)
  1. St Bartholomew's and the Royal London NHS Trust
  2. St Bartholomew's and the Royal London NHS Trust
  3. St Bartholomew's and the Royal London NHS Trust

    Abstract

    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.

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