基于人群的软组织血管肉瘤长期随访:特征、治疗结果和预后因素。
A Population-Based Long-Term Follow-Up of Soft Tissue Angiosarcomas: Characteristics, Treatment Outcomes, and Prognostic Factors.
发表日期:2024 May 11
作者:
Christina Enciso Holm, Mathias Ørholt, Maj-Lis Talman, Kiya Abebe, Andrea Thorn, Thomas Baad-Hansen, Michael Mørk Petersen
来源:
Burns & Trauma
摘要:
血管肉瘤是一种罕见的侵袭性且未被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,基于证据的治疗指南尚未制定。广泛切除手术目前被认为是治疗的基石。在 2000 年至 2017 年丹麦国家健康登记册中确定的基于人群的队列中,本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%。 154 名患者的多变量 Cox 模型显示,年龄超过 60 岁和转移与较差的总体生存率独立相关。皮肤肿瘤、手术和阴性切除边缘与总生存率的改善独立相关。辅助肿瘤治疗并不能改善总体生存率、转移风险或复发风险。阴性切缘与较低的复发和转移风险无关。我们的结论是,尽管广泛切除术后的生存率有所提高,但总体生存率仍然很低。
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.