Uveal melanoma: Estimating prognosis

Abstract

Uveal melanoma is the most common primary malignant tumor of the eye in adults, predominantly found in Caucasians. Local tumor control of uveal melanoma is excellent, yet this malignancy is associated with relatively high mortality secondary to metastasis. Various clinical, histopathological, cytogenetic features and gene expression features help in estimating the prognosis of uveal melanoma. The clinical features associated with poor prognosis in patients with uveal melanoma include older age at presentation, male gender, larger tumor basal diameter and thickness, ciliary body location, diffuse tumor configuration, association with ocular/oculodermal melanocytosis, extraocular tumor extension, and advanced tumor staging by American Joint Committee on Cancer classification. Histopathological features suggestive of poor prognosis include epithelioid cell type, high mitotic activity, higher values of mean diameter of ten largest nucleoli, higher microvascular density, extravascular matrix patterns, tumor-infiltrating lymphocytes, tumor-infiltrating macrophages, higher expression of insulin-like growth factor-1 receptor, and higher expression of human leukocyte antigen Class I and II. Monosomy 3, 1p loss, 6q loss, and 8q and those classified as Class II by gene expression are predictive of poor prognosis of uveal melanoma. In this review, we discuss the prognostic factors of uveal melanoma. A database search was performed on PubMed, using the terms “uvea,” “iris,” “ciliary body,” “choroid,” “melanoma,” “uveal melanoma” and “prognosis,” “metastasis,” “genetic testing,” “gene expression profiling.” Relevant English language articles were extracted, reviewed, and referenced appropriately.

Immunological aspect of the liver and metastatic uveal melanoma

“…The estimated incidence of UM is 5 per million in the United States, and between 2 to 8 per million in Europe [1] . Despite shared embryologic origin, UM differs from the cutaneous melanoma in biological behavior, epidemiology, prognostic features, and molecular profiles [2,3] .…”

“…Such risk factors include: large tumor size, epithelioid cell type, extrascleral extension, loss of chromosome 3 (monosomy 3), and chromosome 8q amplification [4] . Up to 50% of patients with UM develop metastases within 10 years of diagnosis [1] . UM disseminates homogeneous, as there is no significant lymphatic drainage from the eye.…”

Melanoma da Coroideia

“…1,7,8,11,13,19,21 Estima-se que a taxa de mortalidade do melanoma da coroideia aos 10 anos seja de 50%, mas na presença de doença metastática este costuma ser fatal em média após 6 a 12 meses. 6,8,9,16,21 …”

“…[1][2][3][4][5][6][7] É importante referir que o melanoma intraocular (também designado melanoma da úvea) é considerado uma identidade diferente do melanoma cutâneo e da conjuntiva. O primeiro tem origem nos melanócitos da mesoderme e metastiza por via hemática, em contraste com o tumor da pele e mucosas que tem origem na ectoderme e metastiza, preferencialmente, por via linfática.…”

“…Estima-se uma incidência anual de aproximadamente 4 -10 casos por 1 milhão de habitantes. 1,2,3,6,[8][9][10] É mais frequente na sexta década de vida e, ao contrário do que se observa na maioria das neoplasias, a sua incidência parece decrescer em idades mais avançadas. Distribui-se igualmente em ambos os sexos, com predileção por indivíduos de pele e olhos claros.…”