Uveal melanoma is the most common primary intraocular tumor in adults with a reported annual incidence of 6.3 per million among whites, 0.9 among Hispanic and 0.24 among blacks. Activation of the MAPK pathway caused by somatic mutations in GNAQ or GNA11 seems to be strongly involved in the pathogenesis of uveal melanoma. Local treatments include enucleation or more conservative treatments such as brachytherapy and proton-beam external irradiation. No efficacy of any adjuvant therapy was observed. Locoregional treatments, such as hepatic intra-arterial chemotherapy and immunoembolization have been developed for uveal melanoma liver metastasis. Up to 50% of patients with primary uveal melanoma will ultimately develop distant metastasis, associated to a poor prognosis and no effective therapies. Several drugs, such as bevacizumab, ipilimumab, imatinib and MEK-inhibitors, are currently under investigation as single agents or in combination with chemotherapeutic drugs for the treatment of metastatic uveal melanoma.
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