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

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.

Unilateral Multifocal Choroidal Melanoma.

We report a case of multifocal choroidal melanoma in the same eye, separated in presentation by 20 years. A 57-year-old Caucasian male initially presented with a choroidal melanoma of the right eye that was treated with transpupillary thermotherapy. Due to recurrence, the patient underwent proton beam therapy with subsequent tumor regression. A second small choroidal lesion was noted in the right eye during his surveillance examinations that was closely monitored and demonstrated stable dimensions and features suggestive of a choroidal nevus. Twenty years after his first presentation, the second lesion exhibited accelerated growth with imaging studies indicative of transformation to a distinct choroidal melanoma. The patient underwent a second globe salvage treatment of proton beam therapy. We describe the clinical course, radiographic, and imaging findings of this rare choroidal melanoma.