Nascimento AF, Raut CP, Fletcher CD.
   

Department of Pathology, Brigham and Women's Hospital, Boston, MA 02115, USA.
   

Mammary angiosarcoma is a rare neoplasm, accounting for about 0.05% of all primary malignancies of the breast. It is currently believed that histologic grading of mammary angiosarcomas plays an important role in prognostication. Forty-nine cases of primary angiosarcoma of the breast were retrieved from our files. Clinical details and follow-up information were obtained from referring pathologists and clinicians, and by chart review. All statistics were performed using Fisher exact test and only P<0.05 was considered significant. Recurrence-free survival and overall survival curves were established using Statistica software version 5.5 (StatSoft Inc). All patients were female with ages ranging from 15 to 74 years (mean 41.5, median 40). Peak incidence was between the ages of 30 and 50 years. All tumors examined were located within breast parenchyma with or without minor cutaneous involvement. The right side was more commonly affected than the left side (66% vs. 29.5%). Tumor was bilateral at presentation in 2 cases (4.5%). Tumor size varied from 0.7 to 25 cm (mean 6.7, median 5). Most patients presented with a palpable, painless mass. Two patients had a history of prior radiation treatment for breast carcinoma. Histologically, primary tumors were graded using Rosen's 3-tier system: 17 tumors (35.4%) as low grade, 17 (35.4%) as intermediate grade, and 14 (29.2%) as high grade. Forty-six patients were treated surgically, 11 underwent chemotherapy, and 12 patients received radiotherapy. Follow-up was available in 41 patients (83.7%, median duration 29 mo). Ten patients (24.4%) showed evidence of local recurrence within 11 to 60 months (median 36) after diagnosis. Twenty-four patients (58.5%) thus far have developed metastases, which were most commonly to lung, liver, skin, and bone. Time interval between diagnosis and metastasis ranged from 2 to 144 months (median 34). Eighteen patients (44%) so far have died of disease and 1 died of presumably disseminated breast carcinoma. Five patients (12.2%) are alive with disease and 15 patients (36.6%) are alive with no evidence of disease. Statistical analysis evaluating correlation between tumor grade and size, and rate of local recurrence, metastasis, and death owing to disease showed no significant difference among tumors of different grades. The median recurrence-free and overall survival rates for the entire cohort were 2.8 and 5.7 years, respectively. In conclusion, mammary angiosarcoma is a rare disease that affects relatively younger patients. This tumor seems to have an overall similar clinical course as other types of angiosarcoma arising in skin or soft tissue; it carries a moderate risk of local recurrence, and a high risk of metastasis and death. In this large series, there is no correlation between histologic grade and patient outcome, more in line with angiosarcomas at other sites.
   

乳腺血管肉瘤是罕见肿瘤，约占乳腺原发恶性肿瘤的0.05%。目前认为乳腺血管肉瘤的组织学分级有重要的预后意义。我们回顾分析了49例乳腺原发血管肉瘤。临床详细资料和随访信息来自相关病理学医生和临床医生，通过图表分析。所有统计数据都进行Fisher确切检验并且仅当P<0.05时才认为是有意义的。无复发生存曲线和总体生存曲线用统计软件版本5.5（statsoft公司）建立。所有病人均为女性，年龄范围从15到74岁（平均41.5岁，中位数40岁）。高发期在30岁至50岁之间。所有肿瘤都位于乳腺实质伴或不伴有微小皮肤浸润。右侧通常较左侧易累及（66%vs.29.5%）。肿瘤累及双侧者2例（4.5%）。肿瘤大小范围0.7cm到25cm（平均6.7，中位数5）。大多数病人表现为可触及的、无痛性的肿块。2例病人有先期乳腺癌放射治疗病史。组织学上，原发肿瘤用Rosen3等级系统进行分级：低级别17例（35.4%），中级别17例（35.4%），高级别14例（29.2%）。46例病人手术治疗，11例化疗，12例进行了放射治疗。有效随访病例41例（83.7%，中位持续时间29个月）。10例（24.4%）病人在11至60个月内（中位数36）局部复发。24例病人（58.5%）迄今为止出现远处转移，大部分转移至肺、肝、皮肤和骨。诊断和转移之间的时间间隔范围为2到144个月（中位数34）。迄今为止18例（44%）病人死于该病，1例推测死于乳腺癌播散。5例病人（12.2%）带瘤生存，15例（36.6%）无瘤生存，各组织学级别间的肿瘤致死性无明显差别。无瘤生存期和总体生存期的中位数分别是2.8年和5.7年。总之，乳腺血管肉瘤是相对多发生于年轻患者的罕见肿瘤。该肿瘤总体看来同发生于皮肤和软组织的血管肉瘤有类似的临床过程；它有中等风险的局部复发性，和高风险的远处转移及致死性。在该研究系列中，组织学分级与病人预后间无关联，与其他部位的血管肉瘤较符合。

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