July 2019
Volume 60, Issue 9
Free
ARVO Annual Meeting Abstract  |   July 2019
Delayed enucleation of eyes with advanced intraocular retinoblastoma increases metastatic death
Author Affiliations & Notes
  • Zhao Xun Feng
    University of Ottawa, Ontario, Canada
  • Meirong Wei
    Department of Ophthalmology, Liuzhou Maternity and Child Health Care Hospital, Guangxi, China
  • Guohua Liu
    Department of Ophthalmology, Qilu Children’s Hospital of Shandong University, Shandong, China
  • Carlos Solarte
    Department of Ophthalmology, University of Alberta, Alberta, Canada
  • Bin Li
    Department of Ophthalmology, Beijing Tongren Hospital, China
  • Chengyue Zhang
    Department of Ophthalmology, Beijing Children's Hospital, China
  • Yizhuo Wang
    Department of Ophthalmology, Beijing Tongren Hospital, China
  • Brenda L Gallie
    Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Ontario, Canada
    Departments of Ophthalmology, Medical Biophysics, and Medical Genetics, University of Toronto, Ontario, Canada
  • Jungyang Zhao
    Department of Ophthalmology, Beijing Children's Hospital, China
  • Footnotes
    Commercial Relationships   Zhao Xun Feng, None; Meirong Wei, None; Guohua Liu, None; Carlos Solarte, None; Bin Li, None; Chengyue Zhang, None; Yizhuo Wang, None; Brenda Gallie, None; Jungyang Zhao, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 1324. doi:
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      Zhao Xun Feng, Meirong Wei, Guohua Liu, Carlos Solarte, Bin Li, Chengyue Zhang, Yizhuo Wang, Brenda L Gallie, Jungyang Zhao; Delayed enucleation of eyes with advanced intraocular retinoblastoma increases metastatic death. Invest. Ophthalmol. Vis. Sci. 2019;60(9):1324.

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

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Abstract

Purpose : Advanced intraocular retinoblastoma can be cured by primary enucleation, but delay may increase risk of tumor metastasis. We hypothesized that delay in enucleation over a defined length of time would increase metastatic death.

Methods : Ethics Boards of 29 Chinese Centers, in accordance with the Declaration of Helsinki, approved the retrospective study of 600 children enucleated 2006–2010 for advanced retinoblastoma. Excluded were 46 children who died without metastasis or had bilateral advanced disease.

Results : Studied were 554 enucleated eyes/children (202 Group D International Intraocular Retinoblastoma Classification; 352 Group E). Compared to primary enucleation (275 eyes, Group D/E: <0.1 months median time from diagnosis to enucleation), pre-enucleation treatment delayed enucleation (279 eyes, Groups D: 8.4 months; Group E: 2.8 months, p<0.001). Eyes that received pre-enucleation chemotherapy had less high-risk pathology (pT3/pT4) than primarily enucleated eyes (Group D, p=0.04; Group E, p=0.003).

Of 554 children, 22 died from metastasis (6/22 Group D, 16/22 Group E): 14/22 had pre-enucleation chemotherapy, 11/22 had post-enucleation chemotherapy and 5/22 had both. Pathology stages were 4 pT1, 1 pT2b, 2 pT3a, 4 pT3b, 2 pT3c and 9 pT4.

Disease-specific survival (DSS) was lower with prolonged delay between diagnosis and enucleation (Group D, >3.5 vs <3.5 months, p=0.02; Group E, >2 vs <2 months p=0.02).

Secondarily excluded from analysis of the effect of pre-enucleation chemotherapy cycles on DSS were children who received <1 cycle or had other non-chemo pre-enucleation therapy; 510 eyes were included. Too few children with Group D eyes died for analysis. Of children with Group E eyes, those with 1–3 cycles of pre-enucleation chemotherapy had the same DSS as children who had primary enucleation, but those with >4 cycles had lower DSS than children primarily enucleated (p=0.03). Children with high-risk eyes (pT3/pT4) after pre-enucleation chemotherapy had lower DSS than children with high-risk eyes primarily enucleated (p=0.002).

Conclusions : We show that delay from diagnosis to enucleation >3.5 months (Group D) and >2 months (Group E) increases the risk of death from metastasis.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

 

(A) DSS of children with Group D eyes whose eyes were enucleated <3.5 months and >3.5 months after diagnosis. (B) DSS of children with Group E eyes whose eyes were enucleated <2 months and >2 months after diagnosis.

(A) DSS of children with Group D eyes whose eyes were enucleated <3.5 months and >3.5 months after diagnosis. (B) DSS of children with Group E eyes whose eyes were enucleated <2 months and >2 months after diagnosis.

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