Adjuvant External Beam Radiotherapy Following Enucleation of Eyes With Extraocular Extension From Uveal Melanoma

Abstract

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.

Very late orbital recurrence of choroidal melanoma four decades post enucleation

“…In literature, orbital recurrence of CMM has been reported, and may occur several years post enucleation in about 3% of patients. The percentage ranges from 0% 13 to 18% if extra-scleral extension of the tumor is present at time of enucleation. 11,12 This is in line with our patient, who underwent several procedures before the enucleation: first FNBA, followed by scleral flap biopsy, which probably have caused extra-scleral melanoma cells seeding.…”

“…11,12 On the other hand, a recent paper reported no orbital relapse in 51 patients affected by extra-scleral extension, who underwent enucleation followed by observation or external beam radiotherapy. 13 Orbital recurrences after more than 10 years from enucleation are even rarer. In literature, only few reports described orbital recurrence that occurred between 10 and 40 years after primary diagnosis, 12,14–19 out of these only two cases were reported with a gap of about 40 years between enucleation and recurrence.…”

Large extraocular extension of a choroidal melanoma with orbital inflammation

“…Management of choroidal melanoma with extraocular extension varies depending on size of EOE and institutional preference and includes globe-preserving therapy, enucleation, modified enucleation, and orbital exenteration with or without adjunctive therapy. 7 , 9 Adjuvant therapy is often used for cases of large EOE or EOE with incomplete resection, but a survival benefit has not been clearly demonstrated. 9 Given the extent of EOE in our case, globe-preserving therapy was not appropriate, and the patient preferred enucleation to exenteration because of the extent of disfigurement.…”

“… 7 , 9 Adjuvant therapy is often used for cases of large EOE or EOE with incomplete resection, but a survival benefit has not been clearly demonstrated. 9 Given the extent of EOE in our case, globe-preserving therapy was not appropriate, and the patient preferred enucleation to exenteration because of the extent of disfigurement. Considering the risk of local morbidity of adjuvant radiation in the absence of a documented survival benefit and pathology suggestive of complete excision, the patient was observed after enucleation.…”

“…Extraocular extension (EOE) presents in 2–3% of uveal melanoma at the time of diagnosis, is more common in medium and large size tumors, and is typically of limited size. 6 , 7 , 8 , 9 , 10 However, with larger areas of EOE, the best approach for treatment remains controversial. We report the case of a patient with a choroidal melanoma and a large area of extraocular extension, who initially presented with pain and orbital inflammation responsive to corticosteroids, causing diagnostic uncertainty.…”