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

Jonathan E. Lu, Jasmine H. Francis, Ira J. Dunkel, Carol L. Shields, Michael D. Yu, Jesse L. Berry, Kaitlin Kogachi, Alison Skalet, Audra K. Miller, Pranav R. Santapuram, Anthony B. Daniels, David H. Abramson

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish (US)
JournalBritish Journal of Ophthalmology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Retinoblastoma
Neoplasm Metastasis
Mortality
Adjuvant Chemotherapy
Pathology
Survival
Kaplan-Meier Estimate
Complementary Therapies
Optic Nerve
Treatment Failure
Incidence

Keywords

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

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this

Lu, J. E., Francis, J. H., Dunkel, I. J., Shields, C. L., Yu, M. D., Berry, J. L., ... Abramson, D. H. (Accepted/In press). Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017. British Journal of Ophthalmology. https://doi.org/10.1136/bjophthalmol-2018-312915

Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017. / Lu, Jonathan E.; Francis, Jasmine H.; Dunkel, Ira J.; Shields, Carol L.; Yu, Michael D.; Berry, Jesse L.; Kogachi, Kaitlin; Skalet, Alison; Miller, Audra K.; Santapuram, Pranav R.; Daniels, Anthony B.; Abramson, David H.

In: British Journal of Ophthalmology, 01.01.2018.

Research output: Contribution to journalArticle

Lu, JE, Francis, JH, Dunkel, IJ, Shields, CL, Yu, MD, Berry, JL, Kogachi, K, Skalet, A, Miller, AK, Santapuram, PR, Daniels, AB & Abramson, DH 2018, 'Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017', British Journal of Ophthalmology. https://doi.org/10.1136/bjophthalmol-2018-312915
Lu, Jonathan E. ; Francis, Jasmine H. ; Dunkel, Ira J. ; Shields, Carol L. ; Yu, Michael D. ; Berry, Jesse L. ; Kogachi, Kaitlin ; Skalet, Alison ; Miller, Audra K. ; Santapuram, Pranav R. ; Daniels, Anthony B. ; Abramson, David H. / Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017. In: British Journal of Ophthalmology. 2018.
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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.",
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T1 - Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007-2017

AU - Lu, Jonathan E.

AU - Francis, Jasmine H.

AU - Dunkel, Ira J.

AU - Shields, Carol L.

AU - Yu, Michael D.

AU - Berry, Jesse L.

AU - Kogachi, Kaitlin

AU - Skalet, Alison

AU - Miller, Audra K.

AU - Santapuram, Pranav R.

AU - Daniels, Anthony B.

AU - Abramson, David H.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

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