Among patients with clinically apparent radiation retinopathy and/or radiation optic neuropathy, PPCD was lower in the treated eye and correlated with the radiation dose to the optic nerve and the visual acuity. OCTA provides a measure of capillary changes following radiation, and may serve as a quantitative endpoint to address visual prognosis.
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 United States (US) centers.
Methods:
Retrospective chart review at 5 tertiary retinoblastoma centers in
the US for unilateral retinoblastoma patients treated with primary
enucleation, 2007–2017, with ≥1 year of follow-up or treatment
failure.
Results:
Amongst 228 patients (228 eyes), there were 9 metastases (3.9%) and
4 deaths (1.7%). The Kaplan-Meier estimate at 5 years for metastasis-free
survival was 96% (95% confidence interval (CI), 94%−99%), and for
overall survival was 98% (95% CI 96%−100%). All metastases were
evident within 12 months. Histopathology revealed higher risk pathology
(post-laminar optic nerve and/or massive choroidal invasion) in 62 of 228
eyes (27%). Of these higher risk eyes, 39 received adjuvant chemotherapy.
There were 4 subsequent metastases in this higher risk pathology with
adjuvant chemotherapy group, with 3 deaths. Of the 9 overall with
metastases, 7 (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 7 of 9 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 histopathologic findings.
In a patient with ARMD extensively treated with ranibizumab, color fundus photography, fluorescein angiography and SD-OCT images of RAP correlated histopathologically with a paucicellular intraretinal vascular complex.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.