Abstract

UVEAL MELONOMA: CURRENT AND EMERGING TREATMENT OPTIONS

S. D. Rihana*, G. Sandhya Rani, G. Sumanjali, G. Jyothi, G. Chaitanya and D. Rama Brahma Reddy

Abstract

Uveal melanoma (UM) is the most probable intraocular malignancy and arises from melanocytes in the iris, ciliary body, or choroid. primary diagnosis and local treatment is crucial, as survival corresponds with primary tumor size. However, approximately 50% of patients will develop metastatic pathological condition with 6–12 months’ survival from metastatic diagnosis. Genomic analyses have led to the advancement of gene-expression profiles that effectively forecast metastatic progression; unfortunately, no adjuvant therapy has been shown to prolong survival to date. New insights into the molecular biology of UM have discovered recurrent 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 managed an array of new targets for treatment of this disease. Studies are under way with rationally developed regimens targeting these pathways, and innovative agents are under development. We inspect 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, metastasis.