J Retin.  2021 Nov;6(2):65-74. 10.21561/jor.2021.6.2.65.

Uveal Melanoma: A Review of the Literature and Personal Experiences

• Kim YJ
• Lee S
• Lee SC

• KMID: 2552171
• DOI: http://doi.org/10.21561/jor.2021.6.2.65

Abstract

Uveal melanoma is the most common primary intraocular tumor in adults. The annual incidence rate is approximately 0.42 per million people with an increasing trend in South Korea. Approximately 90% of uveal melanomas occur in the choroid, typically presenting as a dome-shaped mass. Radiotherapy is the mainstay of eye-preserving therapy for primary tumors, and plaque brachytherapy is the most common treatment modality for medium-sized tumors. Although primary tumors respond well to local treatment, systemic metastasis is observed in up to 50% of patients. The most commonly affected organ is the liver. Currently, the underlying mechanism of tumorigenesis and metastasis of the uveal melanoma is poorly understood; there is no effective therapy for metastatic tumors. Recent studies have focused on the oncogenic signaling pathways related to driver mutations in the GNAQ, GNA11, PLCB4, and CYSTLR2 genes. However, most clinical studies applying new targeted therapies failed to improve survival. In this review article, we intend to share clinical experience in Korea, and summarize the latest research on molecular characteristics and targeted therapies for uveal melanoma.

Keyword

Brachytherapy; Metastasis; Signaling pathway; Targeted therapy; Uveal melanoma