Uveal Melanoma

Abstract

Uveal melanoma, a malignant proliferation of melanocytes in the uvea, is the most common primary tumor of the eye in adults. The purpose of this review is to discuss the risk factors, clinical presentation, diagnosis, prognosis, and management, while providing information about novel viewpoints and tools for diagnosis and treatment of uveal melanoma. Blue-light exposure, BAP1 polymorphisms, complete monosomy 3, and history of ocular melanocytosis are the greatest risk factors for the development of uveal melanoma. Uveal melanoma can first present either on routine eye exam or upon presentation with flashes, floaters, and visual field defects. Diagnosis of uveal melanoma is made with anterior segment ocular coherence tomography or ultrasound biomicroscopy. Fine-needle biopsy of the lesion often follows diagnosis, with gene expression profiling being the best prognostic indicator for uveal melanoma. The gold standard for treatment of uveal melanoma is plaque brachytherapy. Other treatment possibilities for uveal melanoma include enucleation, charged-particle radiation therapy, transpupillary thermotherapy and photodynamic thermotherapy. Uveal melanoma may be fatal if untreated and subsequently metastasizes. Hopefully, earlier detection methods including the use of circulating tumor cells, circulating tumor DNA, and circulating MicroRNA and subsequent treatment using novel targeted therapies like high-intensity focused ultrasound, iontophoresis and electrochemotherapy would be crucial to improving prognosis in the future.