Successive Uveal Melanomas with Different Gene Expression Profiles in an Eye with Ocular Melanocytosis

Ocular melanocytosis portends a higher risk of uveal melanoma. Multifocal uveal melanoma has been described previously and has been associated with ocular melanocytosis. Historically, histopathology has been used to differentiate tumors; however, molecular profiling now allows for better prognostication and determination of metastatic risk. The present case describes a patient with ocular melanocytosis who developed two sequential uveal melanomas in the same eye separated by 4 years. The uveal melanoma-specific gene expression profile (GEP) testing for the first tumor was class 1A and the second tumor was class 2. While the first tumor had low metastatic risk, the second tumor had a higher risk of metastasis, demonstrating the importance of GEP testing in cases of multifocal disease.