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...

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Published inCancers Vol. 11; no. 11; p. 1780
Main Authors 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
Format Journal Article
LanguageEnglish
Published Switzerland MDPI AG 12.11.2019
MDPI
Subjects
Age
Cancer therapies
Chemotherapy
Confidence intervals
Discordance
Gender
Localization
Lymphedema
Medical prognosis
Metastasis
Pathology
Patients
Radiation therapy
Sarcoma
Surgery
Survival
Survival analysis
Tumors
Ultraviolet radiation
United States > US
Netherlands
angiosarcoma
epidemiology
prognostic factors
pathology review
clinical subtype
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Summary: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.
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Among others; Jos W.R. Meyer, Rijnstate Hospital, Arnhem, The Netherlands and Marieke C.H. Hogenes, LABPON, Hengelo, The Netherlands.
ISSN:2072-6694
2072-6694
DOI:10.3390/cancers11111780