Uveal melanoma(UM)is a rare form of melanoma arising in the uveal tract of the eye.UM has different molecular driving and metastasis patterns as well as different tumor immune microenvironment which are distinct from their more common cutaneous counterparts.These differences lead to poor clinical response to systemic drug therapy,including immune checkpoint inhibitors(ICIs).Treatment of primary UM with radiotherapy,enucleation or other modalities achieves local control in more than 90%of UM patients,although 50%or more eventually develop into distant metastases during disease progression,most commonly in the liver.To date,studies have identified a number of biological features and clinical characteristics of the disease,including the highly prevalent early,oncogenic genomic aberrations in tumor initiation,later recurrent prognostic changes,and immunological features.These advances have contributed to the development of many novel therapies.This review summarizes the biology of UM,management and treatment strategies for primary and recurrent disease,with an emphasis on the current standard treatment for metastatic UM,as well as emerging systemic therapies.
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