Epidemiology, Clinical Presentation and Treatment of Uveal Melanoma

Uveal melanoma represents the most common primary intraocular tumor in adults. However, it remains a relatively infrequent malignancy where large clinical trials are difficult to accomplish. However, during the last couple of years we have witnessed an unprecedented expansion of our understanding of this disease. New genetic and molecular pathways were found to play key roles in the development of uveal melanoma and they represent potential targets for future therapies. At the same time there were some improvements in the delineation of prognostic features as well as treatment options for metastatic disease, with both liver-directed strategies as well as targeted agents. In this review we will try to summarize and update the reader with the most relevant information in terms of its pathogenesis, clinical presentation, prognostic factors– cytogenetic and genetic profiling –, state of the art management of liver only as well as systemic metastatic disease. Finally we will also discuss current ongoing clinical trials as well as future directions in terms of research and clinical investigation.