Abstract：
OBJECTIVE: Angiosarcoma (AS) is a rare malignancy with considerable heterogeneity seen in its aetiology, anatomical location, and clinicopathological behaviour. Diagnosis is often delayed and prognosis poor. The purpose of this study was to perform a retrospective review of all cases of AS over 10 years at a high-volume regional UK referral centre.
METHODS: We reviewed all cases of AS discussed at the sarcoma multidisciplinary meetings of University Hospitals Birmingham NHS Foundation Trust from September 2013 to August 2023. Demographic and clinicopathologic features at diagnosis, approaches to treatment, and outcomes were compared between four AS subtypes.
RESULTS: A total of 130 cases were identified. The median age at diagnosis was 71 years, with the majority being female (78%). The most common AS subtype was radiation-induced AS (RIAS) (n = 72; 55%), followed by primary cutaneous (n = 28; 22%), primary non-cutaneous (n = 25; 19%), and AS secondary to lymphoedema (n = 5; 4%). Metastases were present at diagnosis in 18% of patients. Treatment was with surgery in the majority of patients (71%). The median survival for the cohort was 30 months (95% CI 20-40), although this differed significantly by AS subtype (p < 0.001), ranging from 5 months in primary non-cutaneous AS to 76 months in RIAS.
CONCLUSIONS: RIAS is the most common AS subtype, with surgery the only potentially curative treatment modality. Overall prognosis varies significantly by subtype. An international consensus on classification of AS subtypes is required to allow meaningful comparisons across studies and/or a prospective multi-centre registry.
METHODS: We reviewed all cases of AS discussed at the sarcoma multidisciplinary meetings of University Hospitals Birmingham NHS Foundation Trust from September 2013 to August 2023. Demographic and clinicopathologic features at diagnosis, approaches to treatment, and outcomes were compared between four AS subtypes.
RESULTS: A total of 130 cases were identified. The median age at diagnosis was 71 years, with the majority being female (78%). The most common AS subtype was radiation-induced AS (RIAS) (n = 72; 55%), followed by primary cutaneous (n = 28; 22%), primary non-cutaneous (n = 25; 19%), and AS secondary to lymphoedema (n = 5; 4%). Metastases were present at diagnosis in 18% of patients. Treatment was with surgery in the majority of patients (71%). The median survival for the cohort was 30 months (95% CI 20-40), although this differed significantly by AS subtype (p < 0.001), ranging from 5 months in primary non-cutaneous AS to 76 months in RIAS.
CONCLUSIONS: RIAS is the most common AS subtype, with surgery the only potentially curative treatment modality. Overall prognosis varies significantly by subtype. An international consensus on classification of AS subtypes is required to allow meaningful comparisons across studies and/or a prospective multi-centre registry.
摘要：
目的：血管肉瘤（AS）是一种罕见的恶性肿瘤，其病因具有相当大的异质性，解剖位置,和临床病理行为。诊断常延迟，预后差。这项研究的目的是在英国大批量地区转诊中心对10年以上的所有AS病例进行回顾性审查。
方法：我们回顾了2013年9月至2023年8月在大学医院伯明翰NHS基金会信托的肉瘤多学科会议上讨论的所有AS病例。诊断时的人口统计学和临床病理特征，治疗方法，并比较4种AS亚型的结局.
结果：共确认130例。诊断时的中位年龄为71岁，大多数是女性（78%）。最常见的AS亚型是辐射诱发的AS（RIAS）（n=72；55％），其次是原发性皮肤（n=28；22％），原发性非皮肤（n=25；19％），和AS继发于淋巴水肿(n=5;4%)。18％的患者在诊断时存在转移。大多数患者（71％）接受手术治疗。该队列的中位生存期为30个月(95%CI20-40)，尽管这与AS亚型有显著差异(p<0.001)，从原发性非皮肤AS的5个月到RIAS的76个月不等。
结论：RIAS是最常见的AS亚型，手术是唯一可能治愈的治疗方式。总体预后因亚型而异。需要就AS亚型的分类达成国际共识，以便在研究和/或前瞻性多中心注册表之间进行有意义的比较。
方法：我们回顾了2013年9月至2023年8月在大学医院伯明翰NHS基金会信托的肉瘤多学科会议上讨论的所有AS病例。诊断时的人口统计学和临床病理特征，治疗方法，并比较4种AS亚型的结局.
结果：共确认130例。诊断时的中位年龄为71岁，大多数是女性（78%）。最常见的AS亚型是辐射诱发的AS（RIAS）（n=72；55％），其次是原发性皮肤（n=28；22％），原发性非皮肤（n=25；19％），和AS继发于淋巴水肿(n=5;4%)。18％的患者在诊断时存在转移。大多数患者（71％）接受手术治疗。该队列的中位生存期为30个月(95%CI20-40)，尽管这与AS亚型有显著差异(p<0.001)，从原发性非皮肤AS的5个月到RIAS的76个月不等。
结论：RIAS是最常见的AS亚型，手术是唯一可能治愈的治疗方式。总体预后因亚型而异。需要就AS亚型的分类达成国际共识，以便在研究和/或前瞻性多中心注册表之间进行有意义的比较。
