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Uveal melanoma: epidemiology, etiology, and treatment of primary disease

Authors Krantz BA, Dave N, Komatsubara KM, Marr BP, Carvajal RD

Received 30 August 2016

Accepted for publication 22 November 2016

Published 31 January 2017 Volume 2017:11 Pages 279—289


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Scott Fraser

Benjamin A Krantz,1 Nikita Dave,2 Kimberly M Komatsubara,2 Brian P Marr,3,4 Richard D Carvajal5

1Division of Hospital Medicine, 2Division of Hematology/Oncology, Columbia University Medical Center, 3Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, 4Department of Ophthalmology, Weill Cornell Medical College, 5Division of Hematology/Oncology, Columbia University Medical Center, New York, NY, USA

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, GNAQ and GNA11, 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.

Keywords: uveal melanoma, ocular melanoma, GNAQ, GNA11, MAP kinase, MEK

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