Multiple Uveal Melanoma

Abstract

Introduction: Multiple uveal melanoma is a rare occurrence and includes bilateral melanoma, unilateral multiple/multifocal melanoma, or melanoma with metastasis to the ipsilateral or contralateral eye.

Methods: A chart review of patients diagnosed with uveal melanoma between January 2013 and January 2019 at the Duke University Eye Center Ophthalmic Oncology Service was performed.

Results: Three patients with multiple, sequential melanoma were identified; patient 1 had bilateral choroidal melanoma and patients 2 and 3 had 2 choroidal melanomas occurring in the same eye. In all 3 patients, both the first and sequential choroidal melanomas were treated with I-125 radioactive plaque brachytherapy (PBT). Two patients were found to have developed secondary metastatic uveal melanoma as a presenting sign of systemic metastases. Patient 4, initially treated with PBT, was diagnosed with ipsilateral metastatic choroidal melanoma, also treated with PBT. Patient 5 had right eye enucleation for choroidal melanoma and developed vision-threatening metastasis in the left eye, which was treated with PBRT. None of the patients had history of cancer prior to their first diagnosis. Patients 1 and 5 were tested with a systemic melanoma panel; both were negative for BAP1, but patient 1 had a variant of unknown significance in BRCA2. Patient 3 had oculodermal melanocytosis, an established risk factor of uveal melanoma.

Conclusion: Although rare, the possibility of multiple uveal melanoma does exist. Examination of the treated and contralateral eye on a regular basis is crucial, not only to identify local failure but also new metastases from the primary tumor and additional primary tumors.

Keywords: BAP1; BRCA2; Bilateral choroidal/uveal melanoma; Contralateral metastasis; Interocular metastasis; Intraocular metastasis; Oculodermal melanocytosis; Unilateral multifocal choroidal/uveal melanoma; Unilateral multiple choroidal/uveal melanoma.

Fig. 1

Fundus photographs of patient 1: tumor 1 in the left eye (a) and tumor 2 in the right eye (b), diagnosed concurrently in 2018.

Fig. 2

Patient 2: tumor 1 (a) in the nasal periphery and tumor 2 (b) in the temporal periphery. The fundus photograph (b) also shows a completely regressed tumor 1 nasally with residual chorioretinal scarring in the right eye.

Fig. 3

Patient 3 with ODM. a Slit-lamp photography showing the ocular component. b Fundus photograph showing a regressed tumor 1 at the supratemporal arcade, as well as the newly diagnosed tumor 2 infratemporally in the right eye.

Fig. 4

Fundus photographs of patient 4: tumor 1 (a) diagnosed in January 2011, and tumor 2 (b) diagnosed in January 2018. b Regressed tumor 1 at the inferotemporal arcade and tumor 2 in the far inferior periphery (arrow), both in the left eye. Fundus photographs of Patient 5: tumor 1 (c) in the right eye treated with enucleation in 2010, and tumor 2 (d) in the left eye diagnosed as interocular metastasis.