PDF(Pubmed)
Abstract：
Angiosarcoma is a rare aggressive and understudied soft tissue sarcoma with pending evidence-based treatment guidelines due to varying study cohorts and inconsistent outcome measures. Surgery with wide resection is currently considered to be the cornerstone in management. In a population-based cohort identified from Danish National Health Registers between 2000 and 2017, this study aimed to define prognostic factors in patients with newly diagnosed soft tissue angiosarcoma. Kaplan-Meier survival analysis demonstrated 5-year overall survival of 28%. Competing risk analysis demonstrated cumulative incidence of local recurrence of 30% and metastasis of 43%. Multivariable Cox models among 154 included patients demonstrated age above 60 years and metastasis to be independently associated with worse overall survival. Cutaneous tumors, surgery, and negative resection margin were independently associated with improved overall survival. Adjuvant oncological treatment did not improve overall survival, risk of metastasis, or recurrence. Negative margin was not associated with lower risk of recurrence and metastasis. We conclude that, despite demonstrated improved survival after surgery with wide resection, overall survival remains poor.
摘要：
血管肉瘤是一种罕见的侵袭性和未充分研究的软组织肉瘤，由于不同的研究队列和不一致的结果测量，尚待循证治疗指南。目前，广泛切除的手术被认为是管理的基石。在2000年至2017年从丹麦国家卫生登记册中确定的基于人群的队列中，这项研究旨在确定新诊断的软组织血管肉瘤患者的预后因素。Kaplan-Meier生存分析显示5年总生存率为28%。竞争风险分析表明，局部复发的累积发生率为30％，转移的累积发生率为43％。在154名患者中，多变量Cox模型显示年龄超过60岁，转移与较差的总体生存率独立相关。皮肤肿瘤，手术,阴性切缘与总生存率的提高独立相关。辅助肿瘤治疗并不能提高总生存率。转移的风险，或复发。阴性切缘与较低的复发和转移风险无关。我们的结论是，尽管证明了广泛切除手术后生存率的提高，总体生存率仍然很差。
