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Primary enucleation for group D retinoblastoma in the era of systemic and targeted chemotherapy: the price of retaining an eye
  1. Ido D Fabian1,2,3,
  2. Andrew W Stacey1,2,4,
  3. Kenneth C Johnson2,
  4. Tanzina Chowdhury2,5,
  5. Catriona Duncan2,5,
  6. M Ashwin Reddy2,
  7. Mandeep S Sagoo1,2,6
  1. 1Ocular Oncology Service, Moorfields Eye Hospital, London, UK
  2. 2Retinoblastoma Service, Royal London Hospital, London, UK
  3. 3Ocular Oncology Service, Goldschleger Eye Institute, Sheba Medical Center, Tel-Aviv University, Tel Aviv, Israel
  4. 4Department of Ophthalmology, University of Washington, Seattle, Washington, USA
  5. 5Department of Paediatric Oncology, Great Ormond Street Hospital, London, UK
  6. 6University College London, Institute of Ophthalmology, London, UK
  1. Correspondence to Dr Ido D Fabian, Ocular Oncology Service, Moorfields Eye Hospital, 162 City Road, London EC1V 2PD, UK; didifabian{at}gmail.com

Abstract

Background Chemotherapy is increasingly used as primary treatment for group D retinoblastoma, whereas primary enucleation is considered to have a diminishing role. This study aimed to compare the management course, including number of examinations under anaesthesia (EUAs), of group D patients treated by enucleation versus chemotherapy.

Methods A retrospective analysis of 92 group D patients, of which 40 (37 unilateral) underwent primary enucleation and 52 (17 unilateral) were treated with intravenous chemotherapy. Number of EUAs was compared between the treatment groups with respect to the whole cohort, using univariate and multivariate analysis, and to unilateral cases only.

Results Patients were followed up for a median of 61 months (mean: 66, range: 14–156), in which time primary enucleated patients had on average seven EUAs and chemotherapy-treated patients 21 EUAs (p<0.001). Chemotherapy, young age, bilateral disease, multifocal tumours, familial and germline retinoblastoma were found on univariate analysis to correlate with increased number of EUAs (p≤0.019). On multivariate analysis, however, only treatment type and presentation age were found significant (p≤0.001). On subanalysis of the unilateral cases, patients undergoing primary enucleation had in average seven EUAs, as compared with 16 in the chemotherapy group (p<0.001). Of the 55 unilateral-presenting patients, a new tumour developed in the fellow eye only in a single familial case.

Conclusion Group D patients’ families should be counselled regarding the significant difference in number of EUAs following primary enucleation versus chemotherapy when deciding on a treatment strategy. In this regard, primary enucleation would be most beneficial for older patients with unilateral disease.

  • Posterior Chamber
  • Retina
  • Treatment Surgery

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Footnotes

  • Contributors All authors had substantial contribution to the acquisition and 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.

  • Competing interests None declared.

  • Patient consent Retrospective analysis of anonymised cohort.

  • Ethics approval The Barts Health NHS Trust institutional review board.

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

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