So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020

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Rodrigues, Manuel, de Koning, Leanne, Coupland, Sarah E ORCID: 0000-0002-1464-2069, Jochemsen, Aart G, Marais, Richard, Stern, Marc-Henri, Valente, Andre, Barnhill, Raymond, Cassoux, Nathalie, Evans, Andrew et al (show 40 more authors) , Galloway, Iain, Jager, Martine J, Kapiteijn, Ellen, Romanowska-Dixon, Bozena, Ryll, Bettina, Roman-Roman, Sergio, Piperno-Neumann, Sophie, Alsafadi, Samar, Deacudin, Didier, Leitz, Estelle Frisch Dit, Naguez, Adnan, Nemati, Fariba, Tarin, Malcy, Vivet-Noguer, Raquel, Anelli, Viviana, Mione, Marina, Brouwer, Niels J, Nell, Rogier, van Der Velden, Pieter, Wierenga, Annemijn, Cirri, Erica, Prestat, Antoine, Sengenes, Jennifer, Desjardins, Laurence, Elas, Martyna, Glinkina, Kseniya, Groenewoud, Arwin, Snaar-Jagalska, Ewa, Hascoet, Pauline, Rodrigues, Valeria Pavet, Houkes, Liesbeth, van den Heuvel, Dianne, Kalirai, Helen ORCID: 0000-0002-4440-2576, Traynor, Matthew, Lalanne, Ana, Lantz, Olivier, Mariani, Pascale, Mobuchon, Lenha, Pires, Guilherme Neri and Plomp, Dick (2019) So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020. CANCERS, 11 (7). E1032-.

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Abstract

Despite much progress in our understanding of uveal melanoma (UM) over the past decades, this rare tumour is still often misclassified. Although UM, like other melanomas, is very probably derived from melanocytes, it is drastically different from cutaneous melanoma and most other melanoma subtypes in terms of epidemiology, aetiology, biology and clinical features, including an intriguing metastatic hepatotropism. UM carries distinctive prognostic chromosome alterations, somatic mutations and gene expression profiles, allowing an active tailored surveillance strategy and dedicated adjuvant clinical trials. There is no standard systemic treatment for disseminated UM at present. In contrast to cutaneous melanoma, UMs are not <i>BRAF</i>-mutated, thus curtailing the use of B-Raf inhibitors. Although these tumours are characterised by some immune infiltrates, immune checkpoint inhibitors are rarely effective, possibly due to a low mutation burden. UM patients across the world not only face rare cancer-related issues (e.g., specific management strategies, access to information and to expert centres), but also specific UM problems, which can be exacerbated by the common misconception that it is a subtype of cutaneous melanoma. As a European Consortium dedicated to research on UM and awareness on the disease, "UM Cure 2020" participants urge medical oncologists, pharmaceutical companies, and regulatory agencies to acknowledge UM as a melanoma with specific issues, in order to accelerate the development of new therapies for patients.

Item Type:	Article
Uncontrolled Keywords:	uveal melanoma, cutaneous melanoma, mucosal melanoma, conjunctival melanoma, health policy
Depositing User:	Symplectic Admin
Date Deposited:	03 Oct 2019 08:56
Last Modified:	25 Jan 2024 02:03
DOI:	10.3390/cancers11071032
Open Access URL:	https://doi.org/10.3390/cancers11071032
Related URLs:	Author Publisher
URI:	https://livrepository.liverpool.ac.uk/id/eprint/3056842