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Primary intravenous chemotherapy for group D retinoblastoma: a 13-year retrospective analysis
  1. Ido D Fabian1,2,
  2. Andrew W Stacey1,2,3,
  3. Kenneth P Johnson2,
  4. Zerrin Onadim2,
  5. Tanzina Chowdhury2,4,
  6. Catriona Duncan2,4,
  7. M Ashwin Reddy1,2,
  8. Mandeep S Sagoo1,2,5
  1. 1Ocular Oncology Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK
  2. 2Retinoblastoma Service, Royal London Hospital, London, UK
  3. 3Department of Ophthalmology, University of Washington, Seattle, Washington, USA
  4. 4Paediatric Oncology Department, Great Ormond Street Hospital, London, UK
  5. 5University College London, Institute of Ophthalmology, London, UK
  1. Correspondence to Dr Ido Didi Fabian, Moorfields Eye Hospital, 162 City Road, London EC1V 2PD, UK; didifabian{at}gmail.com

Abstract

Background Eye salvage rate for group D retinoblastoma using intravenous chemotherapy (IVC) as a primary modality is <50%. To report on 13 years' experience with the use of primary IVC for group D retinoblastoma.

Methods A retrospective analysis of 64 group D eyes (52 patients) treated with primary IVC, from 2002 to 2014.

Results The median age at presentation was 11.0 months (mean: 18.6, range: 0.6–144.0), 35 (67%) patients had bilateral disease, 38 (73%) germline disease and 8 (15%) cases were familial. In addition to IVC, patients received a median number of three treatments (mean: 6, range: 0–24), including thermotherapy/cryotherapy, plaque radiotherapy, intra-ophthalmic artery chemotherapy (IAC) and/or intravitreous chemotherapy. External beam radiotherapy (EBRT) was used in five eyes, all of which were eventually enucleated. In a median follow-up time of 55 months (mean: 64, range: 14–156), 63% of eyes were salvaged. By the Kaplan-Meier survival analysis, globe salvage rate was 83%, 70%, 59% and 45% at 1, 3, 5 and 10 years, respectively. There were no cases of metastatic spread from intraocular retinoblastoma and no deaths. IVC-related adverse events included febrile neutropenia in 21 (40%) patients and anaphylactic reaction to carboplatin in 2 (4%), all conservatively resolved. Of the patients receiving IAC, third and sixth nerve palsies were documented in two (10%) and one (5%) eyes, respectively.

Conclusions Primary IVC for group D eyes, with adjuvant treatments as required, was found to be a safe and efficient approach, achieving 63% eye salvage rate, no metastatic spread from intraocular retinoblastoma and no deaths. IAC has now replaced EBRT as a successful salvage treatment.

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Footnotes

  • Twitter Follow Maddy Reddy @childrens eyes

  • Contributors IDF and AWS had substantial contribution to the design of the work, collection and analysis of the data, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. KPJ had substantial contribution to the acquisition, collection and analysis of the work, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. OZ had substantial contribution to the collection and analysis of the data, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. TC and CD had substantial contribution to the conception of the work, collection and analysis of the data, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. MSS and MAR had substantial contribution to the design of the work and analysis of the data, significantly drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Competing interests None.

  • Ethics approval Barts Health NHS Trust Institutional Review Board (number 6622).

  • Provenance and peer review Not commissioned; externally peer reviewed.

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