Management experiences of primary angiosarcoma of breast: a retrospective study from single institute in the People's Republic of China.
Management experiences of primary angiosarcoma of breast: a retrospective study from single institute in the People's Republic of China.
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我国单机构乳腺原发性血管肉瘤治疗经验回顾
DOI：
10.2147/ott.s92769
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发表时间：
2015
影响因子：
4
通讯作者：
Guo XM
中科院分区：
文献类型：
--
作者：
Hu QC;Mei X;Feng Y;Ma JL;Yang ZZ;Shao ZM;Yu XL;Guo XM
摘要乳房原发性血管肉瘤是一种罕见且高度侵袭性的恶性肿瘤。对于这种罕见疾病的最佳治疗方法或预后因素，目前还没有普遍的共识。本研究的目的是探讨PAOB的临床病理特征和治疗经验。我们回顾性分析了复旦大学附属肿瘤医院2000年1月至2014年2月诊断为PAOB的17例连续患者的医疗和病理记录。我们评估了其临床特征、多模式治疗和相关临床结局。本回顾性研究共纳入16例患者（PAOB表现时的中位年龄为33.5岁，范围：19-56岁）。最常见的首发症状为可触及肿块伴或不伴乳房皮肤瘀斑。所有患者均接受手术治疗。中位无病生存期和总生存期分别为9个月和13.6个月。1年和3年无病生存率分别为43.8%和6.3%，OS率分别为93.8%和78.1%。单因素分析显示，组织学分级越高，OS越差（P=0.01）。然而，无论是辅助化疗还是放疗，都没有对我们系列的临床结局做出贡献。PAOB被认为是一种罕见的具有侵袭性特征的乳腺肿瘤。组织学分级和早期转移（诊断后12个月内）与不良预后相关。无论级别如何，辅助治疗均未观察到额外获益。
Primary angiosarcoma of breast (PAOB) is a rare and highly aggressive malignancy. There is no general agreement on optimal treatments or prognostic factors for this orphan disease. The objective of this study was to investigate the clinicopathologic features and management experiences of PAOB. We performed a retrospective review of medical and pathologic records of 17 consecutive patients diagnosed with PAOB between January 2000 and February 2014 at FuDan University Shanghai Cancer Center. We evaluated the clinical characteristics, multimodality treatments, and associated clinical outcomes. A total of 16 patients were included in this retrospective study (median age at PAOB presentation 33.5 years, range: 19–56 years). Palpable tumor with or without breast skin ecchymosis presented as the most common initial symptom. All patients underwent surgery with curative intent. Median disease-free survival and overall survival (OS) were 9 months and 13.6 months, respectively. One-year and 3-year disease-free survival rates were 43.8% and 6.3%, with OS rates of 93.8% and 78.1%, respectively. High histologic grade indicated poorer OS by univariate analysis (P=0.01). However, neither adjuvant chemotherapy nor radiotherapy contributed to clinical outcomes in our series. PAOB is considered as an infrequent breast neoplasm with aggressive characteristics. Histologic grade and early metastasis (within 12 months after diagnosis) are associated with poor prognosis. Regardless of grade, additional benefit was not observed with adjuvant therapy.