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Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas
Angiosarcoma (AS) is a rare sarcoma of endothelial origin, arising spontaneously (primary AS) or after external damage such as radiation therapy or UV exposure (secondary AS). To date, reliable assessment of prognostic factors has proven difficult, due to disease rarity and heterogeneity of study co...
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/PMC6896046/ https://www.ncbi.nlm.nih.gov/pubmed/31726650 http://dx.doi.org/10.3390/cancers11111780 |
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author | Weidema, Marije E. Flucke, Uta E. van der Graaf, Winette T.A. Ho, Vincent K.Y. Hillebrandt-Roeffen, Melissa H.S. Versleijen-Jonkers, Yvonne M.H. Husson, Olga Desar, Ingrid M.E. |
author_facet | Weidema, Marije E. Flucke, Uta E. van der Graaf, Winette T.A. Ho, Vincent K.Y. Hillebrandt-Roeffen, Melissa H.S. Versleijen-Jonkers, Yvonne M.H. Husson, Olga Desar, Ingrid M.E. |
author_sort | Weidema, Marije E. |
collection | PubMed |
description | Angiosarcoma (AS) is a rare sarcoma of endothelial origin, arising spontaneously (primary AS) or after external damage such as radiation therapy or UV exposure (secondary AS). To date, reliable assessment of prognostic factors has proven difficult, due to disease rarity and heterogeneity of study cohorts. Although large registries provide relatively large AS patient series, these cases often lack histological confirmation. This study aimed to analyze AS prognostic factors in a large nationwide cohort of histologically confirmed cases, established through linkage of clinical data from the Netherlands Cancer Registry and pathology data from the Dutch pathology registry (PALGA). All cases were reviewed by an expert pathologist, showing a 16% discordance rate. Multivariable Cox regression survival analysis among 479 confirmed AS patients revealed remarkably poorer overall survival (OS) for primary AS compared to secondary AS (7 vs 21 months, Hazard ratio (HR) = 1.5; 95% confidence interval (CI) = 1.2–1.9). Age above 65 years, male gender, and no surgical treatment also significantly correlated to worse OS. Overall, OS was relatively poor, with a median of 13 months (95% CI = 10–16 months) and 22% five-year survival rate. With this study, we illustrate AS heterogeneity in clinical behavior and show for the first time better survival for secondary AS compared to primary AS. |
format | Online Article Text |
id | pubmed-6896046 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-68960462019-12-23 Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas Weidema, Marije E. Flucke, Uta E. van der Graaf, Winette T.A. Ho, Vincent K.Y. Hillebrandt-Roeffen, Melissa H.S. Versleijen-Jonkers, Yvonne M.H. Husson, Olga Desar, Ingrid M.E. Cancers (Basel) Article Angiosarcoma (AS) is a rare sarcoma of endothelial origin, arising spontaneously (primary AS) or after external damage such as radiation therapy or UV exposure (secondary AS). To date, reliable assessment of prognostic factors has proven difficult, due to disease rarity and heterogeneity of study cohorts. Although large registries provide relatively large AS patient series, these cases often lack histological confirmation. This study aimed to analyze AS prognostic factors in a large nationwide cohort of histologically confirmed cases, established through linkage of clinical data from the Netherlands Cancer Registry and pathology data from the Dutch pathology registry (PALGA). All cases were reviewed by an expert pathologist, showing a 16% discordance rate. Multivariable Cox regression survival analysis among 479 confirmed AS patients revealed remarkably poorer overall survival (OS) for primary AS compared to secondary AS (7 vs 21 months, Hazard ratio (HR) = 1.5; 95% confidence interval (CI) = 1.2–1.9). Age above 65 years, male gender, and no surgical treatment also significantly correlated to worse OS. Overall, OS was relatively poor, with a median of 13 months (95% CI = 10–16 months) and 22% five-year survival rate. With this study, we illustrate AS heterogeneity in clinical behavior and show for the first time better survival for secondary AS compared to primary AS. MDPI 2019-11-12 /pmc/articles/PMC6896046/ /pubmed/31726650 http://dx.doi.org/10.3390/cancers11111780 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 | Article Weidema, Marije E. Flucke, Uta E. van der Graaf, Winette T.A. Ho, Vincent K.Y. Hillebrandt-Roeffen, Melissa H.S. Versleijen-Jonkers, Yvonne M.H. Husson, Olga Desar, Ingrid M.E. Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas |
title | Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas |
title_full | Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas |
title_fullStr | Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas |
title_full_unstemmed | Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas |
title_short | Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas |
title_sort | prognostic factors in a large nationwide cohort of histologically confirmed primary and secondary angiosarcomas |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6896046/ https://www.ncbi.nlm.nih.gov/pubmed/31726650 http://dx.doi.org/10.3390/cancers11111780 |
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