Uveal Melanoma: Epidemiology of Uveal Melanoma and Potential Clusters

Abstract

Uveal melanoma is a rare malignancy affecting approximately 2500 individuals annually in the United States, with an age-adjusted incidence of approximately 5 per million people (Chang et al. Cancer 83:1664–78, 1998). While uveal melanoma comprises 5% of all melanomas, it is the most common primary intraocular malignancy in adults.

Host risk factors for uveal melanoma include fair skin color, non-Hispanic white race, light iris color, and conditions such as dysplastic nevus syndrome, atypical ocular nevi, oculodermal melanocytosis, and germline BAP1 mutations (Nayman et al. Curr Eye Res 42:1085–93, 2017; Hu et al. Am J Ophthalmol 140:612. e1–e8, 2005). Additional genetic germline mutations and systemic disorders are under investigation (Cruz et al. J Clin Oncol 29:e827–e9, 2011; Abdel-Rahman et al. Melanoma Res 21:175–9, 2011; Singh et al. Ophthalmic Genet 16:53–61, 1995).

Environmental risk factors for uveal melanoma have been harder to reliably demonstrate, with conflicting reports of increased uveal melanoma incidence among chemical factory workers, occupational chefs, and arc welders (Nayman et al. Curr Eye Res. 42:1085–93, 2017). The rarity of uveal melanoma has made traditional epidemiologic analysis difficult; however, an investigation into potential uveal melanoma cluster populations in the United States is underway and may provide additional insights in the near future.

This chapter will review the epidemiology of uveal melanoma with special attention to the reported potential for cluster populations identified in the United States.