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‘Uveal melanoma’ is the most common intraocular malignancy in adults and is characterized by aggressive metastases, which mainly develop hematogeneously. Little is known about the factors that predispose patients to develop this disease, although some factors such as skin pigmentation and the presence of multiple cutaneous nevi have been associated with an increased risk to develop uveal melanoma. The intraocular location of uveal melanoma offers excellent opportunities for visualization, and clinical study. While smaller tumors can be treated with eye-preserving therapies, larger tumors sometimes require enucleation of the eye. Since uveal melanomas are typically homogeneous, in contrast other malignancies, comparisons can be made between genetic, immunologic and histological tests on the same tumor. Many countries have excellent registries regarding the survival data of uveal melanoma patients. This has led to a series of studies correlating patient survival to various tumor parameters, such as chromosomal abnormalities, oncogene expression, and immunohistological parameters. The availability of several uveal melanoma cell lines has fostered novel in vitro and in vivo studies that have added to our knowledge about the immunology, genetics, and molecular biology of uveal melanoma. Among other findings, such studies have revealed that that uveal melanoma and cutaneous melanoma are remarkably different diseases.