title: Uveal melanoma: epidemiology, etiology, and treatment of primary disease.
abstract: Uveal melanoma (UM) is the most common intraocular malignancy and arises from melanocytes in the iris, ciliary body, or choroid. Early diagnosis and local treatment is crucial, as survival correlates with primary tumor size. However, approximately 50% of patients will develop metastatic disease with 6-12 months' survival from metastatic diagnosis. Genomic analyses have led to the development of gene-expression profiles that effectively predict metastatic progression; unfortunately, no adjuvant therapy has been shown to prolong survival to date. New insights into the molecular biology of UM have found frequent activating mutations in genes encoding for the G-protein α-subunit,GNAQandGNA11, and improved understanding of the downstream signaling pathways MAPK, PI3K/Akt, and Hippo have afforded an array of new targets for treatment of this disease. Studies are under way with rationally developed regimens targeting these pathways, and novel agents are under development. We review the diagnosis, management, and surveillance of primary UM and the adjuvant therapy trials under way.
authors.affiliation: Division of Hospital Medicine.
authors.forename: Benjamin A
authors.initials: BA
authors.lastname: Krantz
authors.affiliation: Division of Hematology/Oncology, Columbia University Medical Center.
authors.forename: Nikita
authors.initials: N
authors.lastname: Dave
authors.affiliation: Division of Hematology/Oncology, Columbia University Medical Center.
authors.forename: Kimberly M
authors.initials: KM
authors.lastname: Komatsubara
authors.affiliation: Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center; Department of Ophthalmology, Weill Cornell Medical College.
authors.forename: Brian P
authors.initials: BP
authors.lastname: Marr
authors.affiliation: Division of Hematology/Oncology, Columbia University Medical Center, New York, NY, USA.
authors.forename: Richard D
authors.initials: RD
authors.lastname: Carvajal
keywords: GNA11; GNAQ; MAP kinase; MEK; ocular melanoma; uveal melanoma