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Long-term (20-year) real-world outcomes of intravenous chemotherapy (chemoreduction) for retinoblastoma in 964 eyes of 554 patients at a single centre
  1. Carol L Shields1,
  2. Zeynep Bas1,
  3. Sameeksha Tadepalli1,
  4. Lauren A Dalvin1,
  5. Raksha Rao1,
  6. Rachel Schwendeman1,
  7. Sara E Lally1,
  8. Jerry A Shields1,
  9. Amish Shah2,
  10. Ann Leahey2
  1. 1Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, USA
  2. 2Department of Pediatric Oncology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Carol L Shields, Oncology Service, Wills Eye Hospital, Philadelphia, PA 19107, USA; carolshields{at}


Background Intravenous chemotherapy (IVC) remains an important globe salvage therapy for retinoblastoma.

Methods Evaluation of long-term globe salvage at 5, 10, 15 and 20 years following frontline IVC for retinoblastoma.

Results Of 994 eyes, comparison by International Classification of Retinoblastoma group (A vs B vs C vs D vs E) revealed more advanced group with older mean age at presentation (8 vs 7 vs 10 vs 11 vs 15 months, p<0.001). By clinical features, more advanced group demonstrated greater mean tumour diameter (3.2 vs 6.8 vs 9.4 vs 14.3 vs 16.4, p<0.001) and thickness (2.0 vs 3.7 vs 4.4 vs 7.3 vs 9.3, p<0.001), and greater frequency of vitreous seeds ≥1 quadrant (0% vs 0% vs 44% vs 42% vs 57%, p<0.001) and subretinal seeds (0% vs 0% vs 22% vs 65% vs 54%, p<0.001). By outcomes, less advanced group demonstrated greater tumour control (without need for enucleation or external beam radiotherapy (EBRT)) by year 2 (96% vs 91% vs 91% vs 71% vs 32%, p<0.001), and with minimal change up to 20 years. In order to achieve globe salvage, additional intra-arterial chemotherapy (IAC) or plaque radiotherapy was employed by year 2 (5% vs 26% vs 28% vs 27% vs 19%, p<0.001), with little further need up to 20 years. Pinealoblastoma (2%), metastasis (2%) and death (1%) were infrequent.

Conclusion Frontline IVC (plus additional IAC and/or plaque radiotherapy) for retinoblastoma provided complete tumour control for groups A (96%), B (91%), C (91%), D (71%) and E (32%), avoiding enucleation or EBRT and was lasting for up to 20 years.

  • eye (Globe)
  • retina
  • treatment medical
  • vision

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  • Funding Funding was provided in part by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (CLS). CLS has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval The study was conducted in accordance with the Declaration of Helsinki and Institutional Review Board approval was obtained from Wills Eye Hospital.

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

  • Data availability statement No data are available.