Rosalynn R Z Conic1, Giovanni Damiani2, Alice Frigerio3, Sheena Tsai4, Nicola L Bragazzi5, Thomas W Chu6, Natasha A Mesinkovska7, Shlomo A Koyfman8, Nikhil P Joshi8, G Thomas Budd9, Allison Vidimos10, Brian R Gastman11. 1. Department of Surgery, University of Maryland, Baltimore, Maryland; Department of Dermatology and Plastic Surgery, Cleveland Clinic, Cleveland, Ohio; Department of Dermatology, Case Western Reserve University, Cleveland, Ohio. 2. Department of Dermatology, Case Western Reserve University, Cleveland, Ohio. 3. Department of Dermatology, University of Utah School of Medicine, Salt Lake City, Utah. 4. Department of Dermatology, University Hospitals Cleveland Medical Center, Cleveland, Ohio. 5. Department of Health Sciences, School of Public Health, University of Genoa, Genoa, Italy. 6. Department of Dermatology, Wayne State University, Detroit, Michigan. 7. Department of Dermatology, University of California Irvine, Irvine, California. 8. Department of Radiation Oncology, Cleveland Clinic, Cleveland, Ohio. 9. Department of Hematology and Medical Oncology, Cleveland Clinic, Cleveland, Ohio. 10. Department of Surgery, University of Maryland, Baltimore, Maryland. 11. Department of Surgery, University of Maryland, Baltimore, Maryland. Electronic address: gastmab@ccf.org.
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.
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.