Purpose: To report local disease control and all-cause mortality in patients with extraocular extension (EOE) of uveal melanoma (UM) undergoing enucleation followed by observation or external beam radiotherapy (EBRT).Methods: Charts of patients enucleated between January 1 st , 1997 and December 31 st , 2019, with histopathological evidence of EOE of UM were reviewed.
Results:The cohort comprised 51 patients with a mean age of 67 ± 15 years, 22 (43%) of whom underwent adjuvant post-enucleation EBRT. Risk factors for metastasis included presence of epithelioid cells (29/45; 88%), closed loops (20/43; 47%), monosomy 3 (16/25; 64%) and gain of 8q (20/22; 91%). Patients undergoing EBRT had more extensive EOE (median: 5.1 mm vs 2.6 mm, p = 0.008) and surgical excision was less likely to be histologically complete (2/20; 10% vs 14/25; 56%, p = 0.002). Local side effects following EBRT were seen in 64% (14/22). At latest follow up, 59% of patients (30/51) were alive, with a median follow-up of 1.8 years [IQR 2.9, range 0.1 -6.5]. By Kaplan Meier survival analysis, the 5-and 10-year overall survival rates were 56% and 12% respectively. There was no difference in all-cause mortality between those receiving adjuvant EBRT and those who were observed (log rank, p = 0.273). No cases of orbital recurrence were documented.
Conclusions:Orbital EBRT causes significant morbidity. Cases with relatively small EOE undergoing enucleation can be safely observed, without adjuvant EBRT. Multi-center studies are required to better assess the role of EBRT when EOE is more extensive.