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.

1.
Singh AD, De Potter P, Fijal BA, Shields CL, Shields JA, Elston RC: Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis. Ophthalmology 1998;105:195-198.
2.
Honavar SG, Shields CL, Singh AD, et al: Two discrete choroidal melanomas in an eye with ocular melanocytosis. Surv Ophthalmol 2002;47:36-41.
3.
Nissen EJ, Kalirai H, Damato B, Heimann H, Coupland SE: Two distinct uveal melanomas in the same eye. JAMA Ophthalmol 2015;133:1094-1096.
4.
Shields CL, Eagle RC, Ip MS, Marr BP, Shields JA: Two discrete uveal melanomas in a child with ocular melanocytosis. Retina 2006;26:684-687.
5.
Harbour JW: A prognostic test to predict the risk of metastasis in uveal melanoma based on a 15-gene expression profile. Methods Mol Biol 2014;1102:427-440.
6.
Onken MD, Worley LA, Char DH, et al: Collaborative Ocular Oncology Group report number 1: prospective validation of a multi-gene prognostic assay in uveal melanoma. Ophthalmology 2012;119:1596-1603.
7.
Wilkes SR, Robertson DM, Kurland LT, Campbell RJ: Incidence of uveal malignant melanoma in the resident population of Rochester and Olmsted County, Minnesota. Am J Ophthalmol 1979;87:639-641.
8.
Hu DN, Yu GP, McCormick SA, Schneider S, Finger PT: Population-based incidence of uveal melanoma in various races and ethnic groups. Am J Ophthalmol 2005;140:612-617.
9.
Eide N, Garred O, Beiske K, Fodstad O: Bilateral uveal melanomas with different gene expression detected with 7 years interval. Acta Ophthalmol 2016;94:99-102.
10.
Furuta M, Shields CL, Uysal Y, Shields JA: Bilateral primary choroidal melanoma treated with bilateral plaque radiotherapy: a report of three cases. Eur J Ophthalmol 2006;16:879-882.
11.
Hadden PW, Damato BE, McKay IC: Bilateral uveal melanoma: a series of four cases. Eye (Lond) 2003;17:613-616.
12.
Lois N, Shields CL, Shields JA, De Potter P, Ramsey MS: Trifocal uveal melanoma. Am J Ophthalmol 1997;124:848-850.
13.
Tappin MJ, Parsons MA, Sisley K, Rees RC, Rennie IG: Two cases of double melanoma of the uvea. Eye (Lond) 1996;10(Pt 5):600-602.
14.
Cordes FC, Cook RD: Simultaneous bilateral primary ocular malignant melanoma; report of a case. Trans Am Ophthalmol Soc 1949;47:80-92.
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