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.
Department of Dermatology, Case Western Reserve University, Cleveland, Ohio.
J Am Acad Dermatol. 2020 Sep;83(3):809-816. doi: 10.1016/j.jaad.2019.07.024. Epub 2019 Jul 13.
Cutaneous angiosarcoma (CAS) is a rare, malignant tumor of vascular mesenchymal origin accounting for less than 1% of all sarcomas.
To examine epidemiologic trends and outcomes in CAS.
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.
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.
Potential selection and miscoding bias, retrospective nature.
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.
皮肤血管肉瘤（CAS）是一种罕见的、源自血管间充质的恶性肿瘤，占所有肉瘤的比例不足 1%。
研究 CAS 的流行病学趋势和结局。
本回顾性基于人群的研究从监测、流行病学和最终结果数据库中确定了 CAS 患者。计算了年龄、性别和种族标准化发病率（IR）。通过 Kaplan-Meier 曲线和 Cox 比例风险模型评估生存情况。
在 811 名 CAS 患者中，43%有先前的原发性癌症。无先前原发性癌症的 CAS 的 IR 从 1973 年至 1984 年的 5.88/10 万下降到 2005 年至 2014 年的 2.87/10 万。对于有先前原发性癌症的患者，IR 从 1973 年至 1984 年的 0.03/10 万上升到 2005 年至 2014 年的 2.25/10 万。多变量分析显示，年龄大于 70 岁的患者比年龄小于 50 岁的患者死亡风险更高（风险比，2.16；95%置信区间 1.33-3.57；P=0.002），与局限性疾病相比，远处疾病与死亡风险增加相关（风险比，1.50；95%置信区间，1.11-2.03；P=0.008）。接受手术和/或放疗与生存无关。
潜在的选择和分类错误，回顾性研究。
患有其他先前原发性癌症的患者中，CAS 发病率正在上升。目前的治疗策略并未影响生存，这突显了需要更多的治疗选择。
