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So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020
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 i...
Autores principales: | , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678543/ https://www.ncbi.nlm.nih.gov/pubmed/31336679 http://dx.doi.org/10.3390/cancers11071032 |
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author | Rodrigues, Manuel de Koning, Leanne Coupland, Sarah E. Jochemsen, Aart G. Marais, Richard Stern, Marc-Henri Valente, André Barnhill, Raymond Cassoux, Nathalie Evans, Andrew Galloway, Iain Jager, Martine J. Kapiteijn, Ellen Romanowska-Dixon, Bozena Ryll, Bettina Roman-Roman, Sergio Piperno-Neumann, Sophie |
author_facet | Rodrigues, Manuel de Koning, Leanne Coupland, Sarah E. Jochemsen, Aart G. Marais, Richard Stern, Marc-Henri Valente, André Barnhill, Raymond Cassoux, Nathalie Evans, Andrew Galloway, Iain Jager, Martine J. Kapiteijn, Ellen Romanowska-Dixon, Bozena Ryll, Bettina Roman-Roman, Sergio Piperno-Neumann, Sophie |
author_sort | Rodrigues, Manuel |
collection | PubMed |
description | 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 BRAF-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. |
format | Online Article Text |
id | pubmed-6678543 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-66785432019-08-19 So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 Rodrigues, Manuel de Koning, Leanne Coupland, Sarah E. Jochemsen, Aart G. Marais, Richard Stern, Marc-Henri Valente, André Barnhill, Raymond Cassoux, Nathalie Evans, Andrew Galloway, Iain Jager, Martine J. Kapiteijn, Ellen Romanowska-Dixon, Bozena Ryll, Bettina Roman-Roman, Sergio Piperno-Neumann, Sophie Cancers (Basel) Opinion 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 BRAF-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. MDPI 2019-07-22 /pmc/articles/PMC6678543/ /pubmed/31336679 http://dx.doi.org/10.3390/cancers11071032 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Opinion Rodrigues, Manuel de Koning, Leanne Coupland, Sarah E. Jochemsen, Aart G. Marais, Richard Stern, Marc-Henri Valente, André Barnhill, Raymond Cassoux, Nathalie Evans, Andrew Galloway, Iain Jager, Martine J. Kapiteijn, Ellen Romanowska-Dixon, Bozena Ryll, Bettina Roman-Roman, Sergio Piperno-Neumann, Sophie So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 |
title | So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 |
title_full | So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 |
title_fullStr | So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 |
title_full_unstemmed | So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 |
title_short | So Close, yet so Far: Discrepancies between Uveal and Other Melanomas. A Position Paper from UM Cure 2020 |
title_sort | so close, yet so far: discrepancies between uveal and other melanomas. a position paper from um cure 2020 |
topic | Opinion |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678543/ https://www.ncbi.nlm.nih.gov/pubmed/31336679 http://dx.doi.org/10.3390/cancers11071032 |
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