Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
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
_version_ 1783476693956034560
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
work_keys_str_mv AT weidemamarijee prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT fluckeutae prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT vandergraafwinetteta prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT hovincentky prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT hillebrandtroeffenmelissahs prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT versleijenjonkersyvonnemh prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT hussonolga prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas
AT desaringridme prognosticfactorsinalargenationwidecohortofhistologicallyconfirmedprimaryandsecondaryangiosarcomas