Yan Qi, Fernandez Roman A, Elmi Maryam, Gelfond Jonathan, Davies Mark G
Department of Surgery, Long School of Medicine University of Texas Health at San Antonio, San Antonio, TX, United States.
Department of Population Health Sciences, Long School of Medicine University of Texas Health at San Antonio, San Antonio, TX, United States.
Front Surg. 2022 Apr 11;9:819099. doi: 10.3389/fsurg.2022.819099. eCollection 2022.
Angiosarcoma is a rare malignant vascular tumor, and the management and outcome of this disease are not well-described. The aim of this study was to report the incidence, patient demographics, and outcomes of angiosarcoma based on national data.
Data on patients with angiosarcoma were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Inverse probability treatment weights (IPTW) were used to assess the survival benefit of operation with additional chemo or radiation therapy compared to operation alone. These variables were further compared against patients who did not receive an operation despite being initially offered one. Cox regression was used to assess survival. Statistical analyses were performed on RStudio.
For this study, 5,135 patients (46% men; median age 69, range 0-102) with angiosarcoma were identified in the SEER database between 1975 and 2016. The age-adjusted incidence rate was 1-4%. Patients were mostly non-Hispanic Caucasian (75.4%). The average tumor size was 4.7 cm, range (.1-98.9). Tumor grades were high at presentation (Grade III 17.2, Grade IV 19, and unknown 50.6%), but half were considered localized tumors. Most patients underwent an operation (66.1%). In 5.6% of patients, the operation was recommended but not performed. The overall 5-year survival was 26.7% (95% CI 25.4-28.1%). IPTW with adjusted Cox proportional hazard model demonstrated worse survival, showing that operation compared to no operation and operation with chemo/radiation compared to operation alone had worse survival between months 0 and 25 but had improved survival after month 25.
The incidence of angiosarcoma is low and long-term survival is poor. Multimodal therapy in the form of neoadjuvant or adjuvant chemo/radiation therapy offers significant long-term survival benefits over operation alone.
血管肉瘤是一种罕见的恶性血管肿瘤，关于这种疾病的治疗和预后描述尚不充分。本研究的目的是基于全国数据报告血管肉瘤的发病率、患者人口统计学特征及预后。
血管肉瘤患者的数据来自监测、流行病学和最终结果（SEER）数据库。采用逆概率处理权重（IPTW）来评估与单纯手术相比，联合化疗或放疗的手术的生存获益。将这些变量与尽管最初被建议手术但未接受手术的患者进行进一步比较。采用Cox回归评估生存率。在RStudio上进行统计分析。
在本研究中，1975年至2016年期间在SEER数据库中识别出5135例血管肉瘤患者（46%为男性；中位年龄69岁，范围0 - 102岁）。年龄调整后的发病率为1 - 4%。患者大多为非西班牙裔白人（75.4%）。平均肿瘤大小为4.7 cm，范围（0.1 - 98.9）。肿瘤分级在初诊时较高（III级17.2%，IV级19%，不明50.6%），但一半被认为是局限性肿瘤。大多数患者接受了手术（66.1%）。5.6%的患者被建议手术但未进行。总体5年生存率为26.7%（95%可信区间25.4 - 28.1%）。IPTW联合调整后的Cox比例风险模型显示生存率较差，表明与未手术相比，手术以及与单纯手术相比，手术联合化疗/放疗在0至25个月期间生存率较差，但在25个月后生存率有所提高。
血管肉瘤发病率低，长期生存率差。新辅助或辅助化疗/放疗形式的多模式治疗比单纯手术具有显著的长期生存获益。
