A 93-year-old female presented with a suspicious conjunctival lesion in her left eye. Ocular history was significant for posterior chamber lenses in both eyes, status post YAG laser OD and posterior capsule opacification OS. She is being treated for bilateral macular degeneration, with significant vision loss OD. She had a bout of iritis OD two years prior and is currently taking hydroxychloroquine for rheumatoid arthritis. 

Her entering visual acuities were counting fingers at one foot in the right eye and 20/30-2 in the left eye.

On exam, her left eye was noted to have a 2mm x 1.4mm deeply melanotic perilimbal lesion at 7:30 with no overlying vascularity. The iris showed an inferior sectoral melanotic nevus extending from the periphery to the pupillary edge with distortion of the pupil. She also had melanotic lesions in the peripheral iris. 

Gonioscopy revealed pigmented lesions invading the angle in the temporal, nasal and inferior quadrants. With echography the lesions were highly reflective, solid and involving almost all the posterior iris surface, with extensive inferior angle involvement. Her cornea showed mutton fat keratic precipitates.

Click image to enlarge.

Management options for the iris melanoma (brachytherapy, enucleation, observation) were discussed at length. Considering her age, unilateral vision and risk of vision loss from intervention, she opted to observe without treatment but will consider a metastatic work up.

Uveal melanoma is the most common intraocular tumor (incidence is 1:100,000).1 Iris melanoma is largely a clinical diagnosis made with biomicroscopy; echography and anterior segment OCT are also helpful. 

The Collaborative Ocular Melanoma Study found 34% of patients with uveal melanoma will develop metastatic disease within 10 years of diagnosis and 80% of those with metastatic disease will die within one year.2 Chemotherapy, immunotherapy and small-molecule biologics have not improved survival rates. There is no standard of care treatment for uveal metastasis. Primary iris melanomas are most commonly treated by enucleation or radiation; both have similar survival outcomes.

1. Schank T, Hassel JC. Immunotherapies for the treatment of uveal melanoma—history and future. Cancers. 2019;11(8):1048.

2. Diener-West M, Reynolds SM, Agugliaro DJ, et al. Development of metastatic disease after enrollment in the COMS trials for treatment of choroidal melanoma: collaborative ocular melanoma Study Group report No. 26. Arch Ophthalmol. 2005;123:1639–43.