Necrotic Uveal Melanoma Mimics Orbital Cellulitis: A Review

Abstract

<b><i>Background:</i></b> Uveal melanoma is the most common primary intraocular malignancy in adults, often resulting in painless vision loss. We report a case of necrotic uveal melanoma presenting with orbital inflammation mimicking orbital cellulitis and present a comprehensive review of the literature and tabulation of reported cases. <b><i>Summary:</i></b> Our review found 44 published reports of spontaneously necrotic uveal melanoma involving 55 patients. Of these reports, 26 patients (47%) presented with orbital cellulitis. Presenting symptoms of necrotic uveal melanoma with orbital cellulitis included proptosis (82.8%), pain (80.7%), vision loss (61.5%), and restricted extraocular movements (46.2%). <b><i>Key Messages:</i></b> Uveal melanoma can rarely mimic orbital cellulitis. Autoinfarction and tumor necrosis causes secondary orbital inflammation. Intraocular malignancy must remain in the differential for patients with orbital inflammation and vision loss.

Large extraocular extension of a choroidal melanoma with orbital inflammation

“… 5 Tumor necrosis likely occurs from auto-infarction or following growth of the choroidal tumor with insufficient blood supply resulting in ischemia and cellular death. 11 In our case, necrosis of the extraocular component of the lesion likely led to orbital inflammation, explaining the atypical presentation with eyelid edema, chemosis, pain and diplopia. The present case was further complicated by the initial decrease in tumor size following systemic corticosteroid administration.…”

“…Necrotic choroidal tumors have been previously reported to result in scleritis, neovascular glaucoma, choroidal detachment, and orbital inflammation among other inflammatory sequalae. 11 , 12 In previously published reports, this most typically occurs in larger choroidal tumors. 4 Necrotic tumors and the resulting inflammatory sequalae can result in atypical presentations.…”