Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions.
Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions.
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DOI：
10.1097/coc.0b013e31827defa1
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发表时间：
2014-12
期刊：
影响因子：
--
通讯作者：
Ferrari S
中科院分区：
文献类型：
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作者：
Palmerini E;Maki RG;Staals EL;Alberghini M;Antonescu CR;Ferrari C;Ruggieri P;Mavrogenis A;Bertoni F;Cesari M;Paioli A;Marchesi E;Picci P;Ferrari S
骨血管肉瘤是一种罕见的高度恶性血管肿瘤。关于这种肿瘤患者的治疗和结果的文献有限。我们进行了一项两机构的回顾性研究，以分析骨血管肉瘤患者的治疗和生存情况。我们回顾了1980-2009年间收治的原发性骨血管肉瘤的组织学诊断病例。对人口学细节、组织学、治疗和存活率进行了综述。男性38人，女性22人(中位年龄54岁)。大多数病变发生在股骨和骨盆。有24名患者(40%)在发病时被诊断为转移性疾病。43例患者接受了手术，其中30例获得手术完全缓解(SCR)。放疗(RT)17例，化疗(CT)13/35例，转移性肿瘤15/22例。5年总生存率(OS)为20%，局限型患者为33%，转移型患者为0。据报道，获得SCR的患者的5年OS(46%)高于未达到SCR的患者(0%)。在非转移性患者中，在SCR和以顺铂、阿霉素和异环磷酰胺为基础的辅助化疗后，观察到了改善生存率的趋势。15名患者因转移而接受化疗。记录了13名可评价患者的两个RECIST部分反应(紫杉醇[n=1]和阿霉素[n=1])。2例病情稳定。彻底的手术切除是决定预后的关键。转移性疾病或无法切除的疾病患者的存活率非常低。在转移的环境中观察到紫杉烷和蒽环类药物的活性，值得进一步评估。
Angiosarcoma of bone is a rare high-grade malignant vascular tumor. The literature regarding treatment and outcome of patients with this tumor is limited. We performed a two-institutional retrospective study to analyze treatment and survival of patients with angiosarcoma of bone. We reviewed patients with the histological diagnosis of primary angiosarcoma of bone treated from 1980 to 2009. Demographic details, histology, treatment and survival were reviewed. 38 men, 22 women (median age 54 years). Most lesions occurred in the femur and the pelvis. Metastatic disease at presentation was diagnosed in 24 patients (40%). Forty-three patients underwent surgery, with 30 of them achieving surgical complete remission (SCR). Radiotherapy (RT) was applied to 17 patients, and chemotherapy (CT) to 13/35 and 15/22 patients with localized and metastatic disease, respectively. The 5-year overall survival (OS) was 20%: 33% for patients with localized disease and 0% for metastatic patients. Higher 5-year OS was reported for patients who achieved SCR (46%) than for those who did not (0%). In non-metastatic patients, a trend towards improved survival was observed after SCR and adjuvant chemotherapy based on cisplatin, doxorubicin and ifosfamide. Fifteen patients received chemotherapy for metastases. Two RECIST partial responses of 13 evaluable patients were documented (paclitaxel [n=1] and doxorubicin [n=1]). Stable disease was observed in 2 patients. Complete surgical resection is essential for outcome. Survival of patients with metastatic or unresectable disease is very poor. Activity of taxanes and anthracycline was observed in the metastatic setting and merits further evaluation.