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Journal of the American Academy of Dermatology
Sous presse. Epreuves corrigées par l'auteur. Disponible en ligne depuis le vendredi 31 janvier 2020
Doi : 10.1016/j.jaad.2019.07.024
accepted : 9 July 2019
Incidence and outcomes of cutaneous angiosarcoma: A SEER population-based study
 

Rosalynn R.Z. Conic, MD, PhD a, b, c, Giovanni Damiani, MD c, Alice Frigerio, MD, PhD d, Sheena Tsai, MD, MPH e, Nicola L. Bragazzi, MD f, Thomas W. Chu, MD g, Natasha A. Mesinkovska, MD h, Shlomo A. Koyfman, MD i, Nikhil P. Joshi, MD i, G. Thomas Budd, MD j, Allison Vidimos, MD a, Brian R. Gastman, MD a,
a Department of Surgery, University of Maryland, Baltimore, Maryland 
b Department of Dermatology and Plastic Surgery, Cleveland Clinic, Cleveland, Ohio 
c Department of Dermatology, Case Western Reserve University, Cleveland, Ohio 
d Department of Dermatology, University of Utah School of Medicine, Salt Lake City, Utah 
e Department of Dermatology, University Hospitals Cleveland Medical Center, Cleveland, Ohio 
f Department of Health Sciences, School of Public Health, University of Genoa, Genoa, Italy 
g Department of Dermatology, Wayne State University, Detroit, Michigan 
h Department of Dermatology, University of California Irvine, Irvine, California 
i Department of Radiation Oncology, Cleveland Clinic, Cleveland, Ohio 
j Department of Hematology and Medical Oncology, Cleveland Clinic, Cleveland, Ohio 

Correspondence to: Brian R. Gastman, MD, 9500 Euclid Ave, Cleveland, OH 44195.9500 Euclid AveClevelandOH44195
Abstract
Background

Cutaneous angiosarcoma (CAS) is a rare, malignant tumor of vascular mesenchymal origin accounting for less than 1% of all sarcomas.

Objective

To examine epidemiologic trends and outcomes in CAS.

Methods

In this retrospective, population-based study, patients with CAS were identified from the Surveillance Epidemiology and End Results database. Age, sex, and race-standardized incidence rates (IRs) were calculated. Survival was assessed with Kaplan-Meier curves and Cox proportional hazards models.

Results

Of 811 patients with CAS, 43% had a prior primary cancer. CAS IR for patients without prior primary cancers dropped from 5.88 per 100,000 in 1973 to 1984 to 2.87 per 100,000 in 2005 to 2014. In those with prior primary cancers, IR rose from 0.03 per 100,000 in 1973 to 1984 to 2.25 per 100,000 in 2005 to 2014. On multivariate analysis, patients older than 70 years of age had a higher risk of death compared with those younger than 50 years (hazard ratio, 2.16; 95% confidence interval 1.33-3.57; P  = .002), and distant disease was associated with increased risk of death compared with localized disease (hazard ratio, 1.50; 95% confidence interval, 1.11-2.03; P  = .008). Receipt of surgery and/or radiation therapy was not associated with survival.

Limitations

Potential selection and miscoding bias, retrospective nature.

Conclusion

CAS rates are rising among those with other prior primary cancers. Survival is not affected by current therapeutic strategies, highlighting the need for additional treatment options.

The full text of this article is available in PDF format.

Key words : angiosarcoma, cutaneous, disease-specific survival, incidence, outcomes, SEER

Abbreviations used : CAS, CI, IR, RT, SEER



 Funding sources: Dr Conic is supported by National Institute of Arthritis and Musculoskeletal and Skin Diseases grant 5 T32 AR 7569-23.
 Conflicts of interest: None disclosed.
 Reprints not available from the authors.



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