RT Journal Article
SR Electronic
T1 Metastases and death rates after primary enucleation of unilateral retinoblastoma in the USA 2007–2017
JF British Journal of Ophthalmology
JO Br J Ophthalmol
FD BMJ Publishing Group Ltd.
SP 1272
OP 1277
DO 10.1136/bjophthalmol-2018-312915
VO 103
IS 9
A1 Jonathan E Lu
A1 Jasmine H Francis
A1 Ira J Dunkel
A1 Carol L Shields
A1 Michael D Yu
A1 Jesse L Berry
A1 Kaitlin Kogachi
A1 Alison H Skalet
A1 Audra K Miller
A1 Pranav R Santapuram
A1 Anthony B Daniels
A1 David H Abramson
YR 2019
UL http://bjo.bmj.com/content/103/9/1272.abstract
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.