Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
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
_version_ 1783441125948784640
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
work_keys_str_mv AT rodriguesmanuel socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT dekoningleanne socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT couplandsarahe socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT jochemsenaartg socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT maraisrichard socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT sternmarchenri socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT valenteandre socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT barnhillraymond socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT cassouxnathalie socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT evansandrew socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT gallowayiain socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT jagermartinej socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT kapiteijnellen socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT romanowskadixonbozena socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT ryllbettina socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT romanromansergio socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT pipernoneumannsophie socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020
AT socloseyetsofardiscrepanciesbetweenuvealandothermelanomasapositionpaperfromumcure2020