Department of Surgery, Sunderby Hospital, Luleå, Sweden.
Department of Surgery and Perioperative sciences/Surgery, Umeå University, Umeå, Sweden.
Br J Surg. 2023 Nov 9;110(12):1850-1856. doi: 10.1093/bjs/znad290.
Breast angiosarcoma is a rare disease mostly observed in breast cancer (BC) patients who have previously received radiotherapy (RT). Little is known about angiosarcoma aetiology, management, and outcome. The study aim was to estimate risk and to characterize breast angiosarcoma in a Swedish population-based cohort.
The Swedish Cancer Registry was searched for breast angiosarcoma between 1992 and 2018 in three Swedish healthcare regions (population 5.5 million). Information on previous BC, RT, management, and outcome were retrieved from medical records.
Overall, 49 angiosarcomas located in the breast, chest wall, or axilla were identified, 8 primary and 41 secondary to BC treatment. Median age was 51 and 73 years, respectively. The minimum latency period of secondary angiosarcoma after a BC diagnosis was 4 years (range 4-21 years). The cumulative incidence of angiosarcoma after breast RT increased continuously, reaching 1.4‰ after 20 years. Among 44 women with angiosarcoma treated by surgery, 29 developed subsequent local recurrence. Median recurrence-free survival was 3.4 and 1.8 years for primary and secondary angiosarcoma, respectively. The 5-year overall survival probability for the whole cohort was 50 per cent (95 per cent c.i., 21 per cent-100 per cent) for primary breast angiosarcoma and 35 per cent (95 per cent c.i., 23 per cent-54 per cent) for secondary angiosarcoma.
Breast angiosarcoma is a rare disease strongly associated with a history of previous BC RT. Overall survival is poor with high rates of local recurrences and distant metastasis.
乳腺血管肉瘤是一种罕见疾病，主要发生在既往接受过放射治疗（RT）的乳腺癌（BC）患者中。关于血管肉瘤的病因、治疗和预后知之甚少。本研究旨在评估瑞典人群中发生乳腺血管肉瘤的风险，并对其进行特征描述。
在瑞典三个医疗保健地区（人口 550 万），从 1992 年至 2018 年期间，通过瑞典癌症登记处搜索乳腺血管肉瘤病例。从病历中检索到关于既往 BC、RT、治疗和预后的信息。
共发现 49 例位于乳房、胸壁或腋窝的血管肉瘤，其中 8 例为原发性，41 例为 BC 治疗后发生。中位年龄分别为 51 岁和 73 岁。继发性血管肉瘤在 BC 诊断后的最小潜伏期为 4 年（范围 4-21 年）。乳腺 RT 后血管肉瘤的累积发病率持续增加，在 20 年后达到 1.4‰。在接受手术治疗的 44 例血管肉瘤患者中，29 例发生局部复发。原发性和继发性血管肉瘤的无复发生存中位数分别为 3.4 年和 1.8 年。整个队列的 5 年总生存率为原发性乳腺血管肉瘤 50%（95%置信区间，21%-100%）和继发性血管肉瘤 35%（95%置信区间，23%-54%）。
乳腺血管肉瘤是一种罕见疾病，与既往 BC RT 史密切相关。总体生存率较差，局部复发和远处转移率较高。
