June 2023
Volume 64, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2023
Pre-enucleation chemotherapy for retinoblastoma masks high-risk optic nerve invasion
Author Affiliations & Notes
  • Zhao Xun Feng
    Department of Ophthalmology, University of Ottawa, Ottawa, Ontario, Canada
  • Junyang Zhao
    Pediatric Oncology Center, Beijing Children's Hospital Capital Medical University, Beijing, China
  • Nan Zhang
    Department of Pathology, Beijing Children's Hospital Capital Medical University, Beijing, China
  • Mei Jin
    Department of Medical Oncology, Beijing Children's Hospital Capital Medical University, Beijing, China
  • Brenda L Gallie
    Department of Ophthalmology and Visual Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada
  • Footnotes
    Commercial Relationships   Zhao Xun Feng None; Junyang Zhao None; Nan Zhang None; Mei Jin None; Brenda Gallie None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2023, Vol.64, 1268. doi:
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    • Get Citation

      Zhao Xun Feng, Junyang Zhao, Nan Zhang, Mei Jin, Brenda L Gallie; Pre-enucleation chemotherapy for retinoblastoma masks high-risk optic nerve invasion. Invest. Ophthalmol. Vis. Sci. 2023;64(8):1268.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : Increasing success with salvage of retinoblastoma eyes has encouraged trend toward primary chemotherapy instead of primary enucleation. Post-laminar optic nerve invasion (PLONI) observed on pathology after enucleation correlates with high risk of extraocular extension into brain. Pre-enucleation chemotherapy may mask high-risk PLONI, misguiding management and compromising survival.

Methods : Reviewed were children enucleated at 29 Chinese treatment centers demonstrating any degree of intra-laminar or PLONI or tumor at transected optic nerve end. Children with other concomitant high-risk features were excluded. Clinical staging, pre-enucleation treatment, pathology features, adjuvant treatment, times from diagnosis to enucleation, death, and last follow-up were reviewed.

Results : Of 1933 eyes diagnosed with retinoblastoma between 2012 to 2017, 1063 eyes had enucleation: 192 eyes (192 children) had intra-laminar (71), PLONI (101) or tumor at transected optic nerve (20). With mean follow-up 5.3 years, 19 children died, with 16 deaths related to metastatic relapse from enucleated eye (analyzed for cause-specific survival, CCS). Excluded were deaths from pineoblastoma (1), brain hemorrhage (1) or secondary leukemia (1). 5-year overall survival and cause-specific survival (CSS) were 90.0% and 91.0% respectively. Children treated with pre-enucleation chemotherapy had inferior survival to those without, if the eye showed intra-laminar (5-year CSS 88.9% v 100%; P = 0.011) or PLONI (5-year CSS 86.1% v 98.5%; P = 0.022) (Kaplan-Meier (KM) analysis). After adjusting for adjuvant chemotherapy (Cox-proportional analysis), survival disparity due to pre-enucleation chemotherapy was no longer significant for intra-laminar ONI (P = 0.504) and remained significant for PLONI (P = 0.045). Children with optic nerve tumor at transected end treated with or without pre-enucleation chemotherapy had no significant difference in survival (41.7% v 75.0%; P = 0.245, KM analysis).

Conclusions : Children with intra-or post-laminar ONI had lower survival if treated with pre-enucleation chemotherapy. However, adjuvant therapy eliminated survival disparity for intra-laminar invasion. This finding suggests pre-enucleation chemotherapy masks high-risk ONI and misguides adjuvant therapy. For retinoblastoma eyes with poor likelihood of successful salvage, prompt enucleation may be lifesaving.

This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.


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