Cohen-Hallaleh R B, Smith H G, Smith R C, Stamp G F, Al-Muderis O, Thway K, Miah A, Khabra K, Judson I, Jones R, Benson C, Hayes A J
The Sarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, London, UK.
Clin Sarcoma Res. 2017 Aug 7;7:15. doi: 10.1186/s13569-017-0081-7. eCollection 2017.
Radiation induced angiosarcoma (RIAS) of the breast is a rare and aggressive complication of radiotherapy. Due to the rarity of this disease, much of the evidence for its management is based on case reports or small retrospective series. We sought to describe the management and outcomes of RIAS in a large single-institution series.
All patients diagnosed with RIAS between January 2000 and January 2014 were identified from an institutional database.
A total of 49 patients were identified. Median age at diagnosis was 72 years (range 51-93). Median time from completion of radiotherapy to diagnosis of RIAS was 7.5 years. Median tumour size at presentation was 5.0 cm (1.5-19.0). The majority of patients presented with localised disease (47, 95.9%). Of these, 35 (74.5%) were suitable for surgery and underwent surgery with curative intent. Twelve patients presented with localised irresectable disease. Of these, 7 received systemic chemotherapy, with a sufficient response to facilitate surgery in 3 patients. Following potentially curative surgery, 2-year local recurrence-free was 55.2%. Survival was significantly prolonged in patients presenting with resectable disease (2-year overall survival 71.1% vs 33.3%, p < 0.001). Tumour size >5 cm was prognostic of distant metastases-free survival and overall survival.
RIAS are rare, aggressive soft-tissue lesions with limited treatment options and high-rates of both local and systemic relapse.
乳腺放射性血管肉瘤(RIAS)是放疗罕见且侵袭性强的并发症。由于该疾病罕见,其治疗的许多证据基于病例报告或小型回顾性系列研究。我们试图在一个大型单机构系列研究中描述RIAS的治疗及预后。
从机构数据库中识别出2000年1月至2014年1月期间所有诊断为RIAS的患者。
共识别出49例患者。诊断时的中位年龄为72岁(范围51 - 93岁)。从放疗结束到诊断为RIAS的中位时间为7.5年。就诊时肿瘤的中位大小为5.0厘米(1.5 - 19.0厘米)。大多数患者表现为局限性疾病(47例,95.9%)。其中,35例(74.5%)适合手术并接受了根治性手术。12例患者表现为局限性不可切除疾病。其中,7例接受了全身化疗,3例有足够反应从而得以进行手术。在进行了可能根治性手术之后,2年局部无复发生存率为55.2%。可切除疾病患者的生存期显著延长(2年总生存率71.1%对33.3%,p < 0.001)。肿瘤大小>5厘米是无远处转移生存期和总生存期的预后指标。
RIAS是罕见、侵袭性强的软组织病变,治疗选择有限,局部和全身复发率高。