Introduction: Angiosarcomas arise from vascular or lymphatic endothelial cells and can develop at any site. Visceral angiosarcomas are aggressive high-grade tumors with a high risk of recurrence, metastasis, and poor survival. Nationwide studies with long-term follow-up are limited, but crucial for understanding this malignancy. This study aimed to describe a national cohort of patients with visceral angiosarcomas and estimate long-term survival, local recurrence, and metastases.Methods: We included all adult patients in Denmark diagnosed with histologically confirmed visceral angiosarcoma from 2000 to 2017. Data were obtained from the Danish Pathology Register and the Danish Sarcoma Database, both providing nationwide and comprehensive records. Additional information on demographics, comorbidities, symptoms, diagnosis, tumor location, treatment, recurrence, and survival were collected from registries and health records.Results: Eighteen patients with visceral angiosarcoma were identified, corresponding to an incidence of one per 5.5 million inhabitants per year. The median age was 56.5 years (IQR: 50–70), and 56% were female. Tumors were most commonly located in the kidney, liver, and thoracic wall. Metastases were present at diagnosis in 17% and developed later in 50%. Surgery was performed in 61%, with R0 resection in 55%. Median overall survival was 249 days (IQR: 121–858), and the 5-year survival rate was 11%. Only one patient (6%) remained alive at long-term follow-up.Conclusions: This Danish nationwide study confirms that visceral angiosarcomas are rare, highly aggressive tumors with a poor prognosis, consistent with international findings. Despite the small cohort, the disease demonstrated significant heterogeneity in anatomical location, metastatic pattern, and treatment approaches.
引言：血管肉瘤起源于血管或淋巴管内皮细胞，可发生于全身任何部位。内脏血管肉瘤是具有高度侵袭性的高级别肿瘤，复发和转移风险高，患者生存预后差。目前针对该恶性肿瘤的全国性长期随访研究有限，但此类研究对于深入理解该疾病至关重要。本研究旨在描述丹麦全国范围内的内脏血管肉瘤患者队列，并评估其长期生存率、局部复发及转移情况。 方法：我们纳入了2000年至2017年间丹麦所有经组织学确诊为内脏血管肉瘤的成年患者。数据来源于丹麦病理学登记库和丹麦肉瘤数据库，这两个数据库均提供全国性、全面的医疗记录。通过医疗登记系统和健康档案，我们进一步收集了患者的人口统计学特征、合并症、临床症状、诊断信息、肿瘤部位、治疗方案、复发情况及生存数据。 结果：共确诊18例内脏血管肉瘤患者，相当于每年每550万居民中发生1例。患者中位年龄为56.5岁（四分位距：50-70岁），其中56%为女性。肿瘤最常见部位为肾脏、肝脏和胸壁。确诊时已有转移的患者占17%，后续发生转移者达50%。61%的患者接受了手术治疗，其中55%实现了R0切除。患者中位总生存期为249天（四分位距：121-858天），5年生存率为11%。长期随访中仅1例患者（6%）存活。 结论：这项丹麦全国性研究证实，内脏血管肉瘤是一种罕见且具有高度侵袭性的肿瘤，预后不良，与国际研究结果一致。尽管研究队列规模较小，但该疾病在解剖部位、转移模式和治疗方法方面表现出显著的异质性。
Visceral Angiosarcoma: A Nationwide Population-Based Study from 2000–2017
肿瘤(癌症)患者之家