Necrotic Uveal Melanoma Mimics Orbital Cellulitis: A Review

Abstract

Background: 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.

Summary: 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%).

Key messages: 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.

Keywords: Necrotic uveal melanoma; Orbital cellulitis; Orbital inflammation; Spontaneous necrosis.

Fig. 1

a, b External photographs of the right eye demonstrating eyelid edema and erythema, conjunctival injection and chemosis, and hyphema at initial presentation. c External photograph two months after initial presentation demonstrating conjunctival injection, shallow anterior chamber, and fibrinoid debris in the anterior chamber without a clear view of the pupil.

Fig. 2

Axial T1 MRI with contrast image demonstrating right eye proptosis, orbital inflammation, intraocular debris and hemorrhage, and an enhancing intraocular mass.

Fig. 3

a ×2 magnification micrograph of the posterior chamber of the globe. From left to right: necrotic tumor, melanin-laden macrophages (melanophages), edematous choroid, and sclera (hematoxylin and eosin stain). b ×60 magnification micrograph of tumor showing “ghosts” of both spindled and epithelioid cells. Pigment is present in the majority of these cells (hematoxylin and eosin stain). c ×60 magnification micrograph of Fontana-Masson stain confirming that the pigment in the cells is melanin.