Abstract：
Angiosarcoma (AS) of the breast is very rare, accounting for 1% of all soft tissue breast tumors. AS may present as primary tumors of the breast or as secondary lesions usually associated with previous radiotherapy. Commonly, secondary AS affects older women (median age 67-71 years) with a clinical history of breast cancer. The preferred site of onset of RIAS is the edge of radiation fields, where radiation doses and tumor necrosis may be heterogeneous, resulting in a DNA damage and instability. Radical surgery is the treatment of choice, but no clear consensus exists on surgical management of breast AS.
We describe an atypical case of relapsed RIAS after radical mastectomy, treated with new surgery and, considering the higher risk of recurrence, subsequent adjuvant chemotherapy with weekly paclitaxel.
The frequency of radiation-induced angiosarcomas (RIAS) after breast-conserving surgery and radiotherapy has been increased to 0.14-0.5% among long survivors. Nevertheless, even if RIAS continues to be prognostically an extremely unfavorable cancer due to a high rate of recurrence, distant spread, and median overall survival (OS) of about 60 months, the benefits of loco-regional breast radiotherapy are clearly higher than the risk in developing angiosarcoma.
We describe an atypical case of relapsed RIAS after radical mastectomy, treated with new surgery and, considering the higher risk of recurrence, subsequent adjuvant chemotherapy with weekly paclitaxel.
The frequency of radiation-induced angiosarcomas (RIAS) after breast-conserving surgery and radiotherapy has been increased to 0.14-0.5% among long survivors. Nevertheless, even if RIAS continues to be prognostically an extremely unfavorable cancer due to a high rate of recurrence, distant spread, and median overall survival (OS) of about 60 months, the benefits of loco-regional breast radiotherapy are clearly higher than the risk in developing angiosarcoma.
摘要：
背景：乳腺血管肉瘤（AS）非常罕见，占全部乳腺软组织肿瘤的1%。AS可表现为乳腺原发性肿瘤或通常与先前放疗相关的继发性病变。通常,继发性AS影响有乳腺癌临床病史的老年女性(中位年龄67~71岁).RIAS发作的首选部位是辐射场的边缘，辐射剂量和肿瘤坏死可能是异质的，导致DNA损伤和不稳定。根治性手术是首选的治疗方法，但对于乳腺AS的外科治疗尚无明确共识.
方法：我们描述了一个在根治性乳房切除术后复发RIAS的非典型病例，接受新手术治疗，考虑到更高的复发风险，随后每周紫杉醇辅助化疗。
结论：在长期存活者中，保乳手术和放疗后放射诱导的血管肉瘤（RIAS）的发生率增加至0.14-0.5%。然而，即使RIAS由于高复发率而在预后上仍然是极其不利的癌症，遥远的传播，中位总生存期（OS）约为60个月，局部区域乳腺放疗的获益明显高于发生血管肉瘤的风险.
方法：我们描述了一个在根治性乳房切除术后复发RIAS的非典型病例，接受新手术治疗，考虑到更高的复发风险，随后每周紫杉醇辅助化疗。
结论：在长期存活者中，保乳手术和放疗后放射诱导的血管肉瘤（RIAS）的发生率增加至0.14-0.5%。然而，即使RIAS由于高复发率而在预后上仍然是极其不利的癌症，遥远的传播，中位总生存期（OS）约为60个月，局部区域乳腺放疗的获益明显高于发生血管肉瘤的风险.
