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Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007–2017
  1. Jonathan E Lu1,
  2. Jasmine H Francis1,2,
  3. Ira J Dunkel3,
  4. Carol L Shields4,
  5. Michael D Yu4,
  6. Jesse L Berry5,
  7. Kaitlin Kogachi5,
  8. Alison H Skalet6,7,
  9. Audra K Miller6,7,
  10. Pranav R Santapuram8,
  11. Anthony B Daniels8,
  12. David H Abramson1,2
  1. 1 Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York City, New York, USA
  2. 2 Department of Ophthalmology, Weill Cornell Medical College, New York City, New York, USA
  3. 3 Department of Pediatrics, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York City, New York, USA
  4. 4 Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
  5. 5 Children’s Hospital Los Angeles & the USC Roski Eye Institute, Keck Medical School of the University of Southern California, Los Angeles, California, USA
  6. 6 Casey Eye Institute, Oregon Health & Science University, Portland, Oregon, USA
  7. 7 Department of Radiation Medicine, Oregon Health & Science University, Portland, Oregon, USA
  8. 8 Department of Ophthalmology and Visual Sciences, Vanderbilt University Medical Center, Nashville, Tennessee, USA
  1. Correspondence to Dr David H Abramson, Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York City, NY 10065, USA; abramsod{at}


Background/aims Enucleation for retinoblastoma is performed less often in the past decade due to increasingly widespread alternative therapies, but enucleation remains an important option. There is a paucity of reports on the current incidence of metastases and metastatic deaths in unilateral retinoblastoma from US centres.

Methods Retrospective chart review at five tertiary retinoblastoma centres in the USA for unilateral retinoblastoma patients treated with primary enucleation, 2007–2017, with >1 year of follow-up or treatment failure.

Results Among 228 patients (228 eyes), there were nine metastases (3.9%) and four deaths (1.7%). The Kaplan-Meier estimate at 5 years for metastasis-free survival was 96% (95% CI, 94% to 99 %), and for overall survival was 98% (95% CI 96% to 100%). All metastases were evident within 12 months. Histopathology revealed higher risk pathology (postlaminar optic nerve and/or massive choroidal invasion) in 62 of 228 eyes (27%). Of these higher risk eyes, 39 received adjuvant chemotherapy. There were four subsequent metastases in this higher risk pathology with adjuvant chemotherapy group, with three deaths. Of the nine overall with metastases, seven (78%) showed higher risk pathology. All metastatic patients were classified as Reese-Ellsworth V and International Classification of Retinoblastoma Groups D or E. Initial metastases presented as orbital invasion in seven of nine cases.

Conclusions Primary enucleation for unilateral retinoblastoma results in a low rate of metastatic death, but is still associated with a 3.9% chance of metastases within a year of enucleation. Most but not all patients who developed metastases had higher risk histopathological findings.

  • epidemiology
  • retina
  • neoplasia
  • child health (paediatrics)

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  • Contributors Conception or design of the work: JEL, JHF, IJD, DHA. Data collection: JEL, JHF, IJD, CLS, MDY, JLB, KK, AHS, AKM, PRS, ABD, DHA. Data analysis and interpretation: JEL, JHF, IJD, DHA. Drafting the article: JEL, JHF, IJD, DHA. Critical revision of the article JEL, JHF, IJD, CLS, MDY, JLB, KK, AHS, AKM, PRS, ABD, DHA. Final approval of the version to be published: JEL, JHF, IJD, CLS, MDY, JLB, KK, AHS, AKM, PRS, ABD, DHA.

  • Funding This work was supported by the Fund for Ophthalmic Knowledge, Inc and MSK Cancer Center Support Grant/Core Grant P30 CA008748 (JEL, JHF, IJD and DHA) . This work was also supported by the National Institutes of Health (NIH) grant P30 EY010572 and by unrestricted departmental funding from Research to Prevent Blindness (AHS and AKM). Additionally this work was supported by the NIH/NEI grant 5K08EY027464-02, a Research to Prevent Blindness Career Development Award and by an unrestricted grant from Research to Prevent Blindness to the Vanderbilt Department of Ophthalmology and Visual Sciences (ABD). None of the funding sources were involved in the study design, execution, analysis or interpretation.

  • Disclaimer All persons who meet authorship criteria are listed as authors, and all authors certify that they have participated sufficiently in the work to take public responsibility for the content, including participation in the concept, design, analysis, writing or revision of the manuscript. Furthermore, each author certifies this material or similar material has not been and will not be submitted to or published in any other publication before its appearance in the British Journal of Ophthalmology.

  • Competing interests No authors have any interests relevant to this study to disclose. Interests outside of the submitted work: JEL, JHF, CLS, MDY, JLB, KK, AKM, PRS and DHA have nothing to disclose. IJD reports other from Apexigen, personal fees from Bayer, grants and personal fees from Bristol-Myers Squibb, personal fees from Celgene, personal fees from Eisai, personal fees from Ipsen, personal fees from Pfizer, grants from Genentech, outside the submitted work. AHS reports personal fees from Castle Biosciences, Inc, outside the submitted work. ABD reports grants from Research to Prevent Blindness, grants from Knights Templar Eye Foundation, grants from Alcon Research Institute, grants from Spectrum Pharmaceuticals, outside the submitted work.

  • Patient consent Not required.

  • Ethics approval Memorial Sloan Kettering Cancer Center.

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

  • Data sharing statement There are not currently any unpublished data available.