Clinical management of uveal melanoma: a comprehensive review with a treatment algorithm

Abstract

Melanoma is a malignancy that originates from the neoplastic proliferation of melanin-producing cells known as melanocytes, which can be primarily found in the skin, ocular region and mucous membranes. Uveal melanoma (UM) is the most frequently occurring non-cutaneous melanoma and is the most common primary intraocular malignancy in adults [1]. The uveal tract, a layer underlying the sclera of the eye, includes the iris, ciliary body and choroid. Around 95% of uveal melanomas arise from the choroidal melanocytes. 1. Epidemiology The worldwide incidence of UM is estimated to be close to 4 to 5 Uveal melanoma (UM), the most frequently occurring non-cutaneous melanoma and most common primary intraocular malignancy in adults, arises from the melanocytes of the choroid in approximately 95% of cases. Prompt diagnosis and treatment is vital as primary tumor size is one of the key factors associated with survival. Despite recent advances in management, more than half of the patients develop metastatic disease which portends poor survival. Currently, treatment options for UM include local resection, enucleation, plaque brachytherapy, and/or particle beam radiotherapy (RT). Enucleation was initially the standard of care in the management of UM, but a shift towards eye-preserving therapeutic choices such as RT and local resection has been noted in recent decades. Plaque brachytherapy, a form of localized RT, is the most popular option and is now the preferred treatment modality for a majority of UM cases. In this review we discuss the etiopathogenesis, clinical presentation and diagnosis of UM and place a special emphasis on its therapeutic options. Furthermore, we review the current literature on UM management and propose a functional treatment algorithm for non-metastatic disease.

Clinical determinants of long-term survival in metastatic uveal melanoma

“…The mean age-adjusted incidence is 5.2 per million [1] and shows a north-to-south decreasing gradient in Europe [2], with the highest current mean incidence of 9.5 per million in Ireland [3]. The treatment approach of the primary tumor depends on the tumor size, patient preference, and tumor localization, most commonly by brachytherapy or enucleation [4,5]. The most important risk factor for the development of metastases is monosomy 3, and at least 40-50% of patients will develop metastases, predominantly to the liver [6,7].…”

Long-Term Outcomes in Uveal Melanoma After Ruthenium-106 Brachytherapy

“…COMS criteria defined small and medium choroidal melanomas as having an apical height of 3 and 3–8 mm, respectively ( 12 ).…”

Importance of Optical Coherence Tomography and Optical Coherence Tomography Angiography in the Imaging and Differentiation of Choroidal Melanoma: A Review

“…SS-OCTA has also been used for the differential diagnosis of choroidal melanomas and other masses, such as nevi, circumscribed choroidal hemangioma, optic disk melanocytoma, retinal astrocytic hamartoma, or metastasis [ 71 , 72 ]. Choroidal nevi, retinal hamartoma, and circumscribed choroidal hemangioma are the only lesions with well-demarcated borders.…”