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Multicenter Study
. 2019 Sep;103(9):1272-1277.
doi: 10.1136/bjophthalmol-2018-312915. Epub 2018 Oct 25.

Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017

Jonathan E Lu  1 , Jasmine H Francis  1   2 , Ira J Dunkel  3 , Carol L Shields  4 , Michael D Yu  4 , Jesse L Berry  5 , Kaitlin Kogachi  5 , Alison H Skalet  6   7 , Audra K Miller  6   7 , Pranav R Santapuram  8 , Anthony B Daniels  8 , David H Abramson  9   2
Affiliations
Multicenter Study

Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017

Jonathan E Lu et al. Br J Ophthalmol. 2019 Sep.

Abstract

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.

Keywords: child health (paediatrics); epidemiology; neoplasia; retina.

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Conflict of interest statement

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.

Figures

Figure 1:
Figure 1:
Kaplan-Meier survival curve of metastasis-free survival over time of follow-up. The dotted line is the 95% confidence interval.
Figure 2:
Figure 2:
Kaplan-Meier survival curve of metastasis-specific survival over time of follow-up. The dotted line is the 95% confidence interval.
See this image and copyright information in PMC

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