Department of Surgery, Pardubice General Hospital, Pardubice, Czech Republic; Faculty of Medicine and Dentistry, Palacký University Olomouc, Olomouc, Czech Republic.
Faculty of Health Studies, Department of Clinical Subspecialities, University of Pardubice, Pardubice, Czech Republic.
Surg Oncol. 2022 May;41:101726. doi: 10.1016/j.suronc.2022.101726. Epub 2022 Feb 16.
Radiation-associated angiosarcoma (RAAS) is a rare and serious complication of breast irradiation. Due to the rarity of the condition, clinical experience is limited and publications on this topic include only retrospective studies or case reports.
All patients diagnosed with RAAS between January 2000 and December 2017 in twelve centers across the Czech Republic and Slovakia were evaluated.
Data of 53 patients were analyzed. The median age at diagnosis was 72 (range 44-89) years. The median latency period between irradiation and diagnosis of RAAS was 78 (range 36-172) months. The median radiation dose was 57.6 (range 34-66) Gy. The whole breast radiation therapy with radiation boost to the tumor bed was the most common radiotherapy regimen. Total mastectomy due to RAAS was performed in 43 patients (81%), radical excision in 8 (15%); 2 patients were not surgically treated due to unresectable disease. Adjuvant chemotherapy followed surgical therapy of RAAS in 18 patients, 3 patients underwent adjuvant radiotherapy. The local recurrence rate of RAAS was 43% and the median time from surgery to the onset of recurrence was 7.5 months (range 3-66 months). The 3-year survival rate was 56%, the 5-year survival rate was only 33%. 46% of patients died during the follow-up period.
The present data demonstrate that RAAS is a rare condition with high local recurrence rate (43%) and mortality (the 5-year survival rate was 33%.). Early diagnosis of RAAS based on biopsy is crucial for treatment with radical intent. Surgery with negative margins constitutes the most important part of the therapy; the role of adjuvant chemotherapy and radiotherapy is still unclear.
放射性血管肉瘤(RAAS)是乳房放疗的一种罕见且严重的并发症。由于这种疾病较为罕见,临床经验有限,因此关于这个主题的出版物仅包括回顾性研究或病例报告。
在捷克共和国和斯洛伐克的 12 个中心,评估了所有在 2000 年 1 月至 2017 年 12 月期间诊断为 RAAS 的患者。
分析了 53 名患者的数据。诊断时的中位年龄为 72 岁(范围为 44-89 岁)。从放疗到 RAAS 诊断的中位潜伏期为 78 个月(范围为 36-172 个月)。中位放疗剂量为 57.6Gy(范围为 34-66Gy)。最常见的放疗方案是全乳房放疗加肿瘤床放疗增强。由于 RAAS,43 名患者(81%)接受了全乳房切除术,8 名患者(15%)接受了根治性切除术;2 名患者由于疾病无法切除而未接受手术治疗。18 名患者在接受 RAAS 手术治疗后接受了辅助化疗,3 名患者接受了辅助放疗。RAAS 的局部复发率为 43%,从手术到复发的中位时间为 7.5 个月(范围 3-66 个月)。3 年生存率为 56%,5 年生存率仅为 33%。46%的患者在随访期间死亡。
目前的数据表明,RAAS 是一种罕见的疾病,局部复发率高(43%),死亡率高(5 年生存率为 33%)。基于活检的 RAAS 早期诊断对于根治性治疗至关重要。切缘阴性的手术是治疗的最重要部分;辅助化疗和放疗的作用仍不清楚。