Fayette J, Martin E, Piperno-Neumann S, Le Cesne A, Robert C, Bonvalot S, Ranchère D, Pouillart P, Coindre J M, Blay J Y
Hospices Civils de Lyon, Université Lyon 1, Hôpital Edouard Herriot, Oncologie Médicale, Pavillon E, Lyon, France.
Ann Oncol. 2007 Dec;18(12):2030-6. doi: 10.1093/annonc/mdm381. Epub 2007 Oct 31.
Angiosarcomas are rare, heterogeneous and a retrospective study was conducted to describe their natural history.
We reviewed 161 files of angiosarcoma treated in three institutions of the French Sarcoma Group from 1980 to 2004. Survival and prognostic factors for survival were analyzed.
Median age was 52 years. Primary sites were the breast (35%), skin (20%) and soft tissues (13%). At initial diagnosis, 31 (19%) had metastases. Surgery was the first treatment in 121 (75%) patients combined with chemotherapy or radiotherapy in 34 and 32, respectively. Ninety (74%) of these 121 patients relapsed, mostly locally (50). With an average time since initial diagnosis of 8.1 years, 123 (76%) patients progressed and 76 (47%) died. Median survival was 3.4 years [95% confidence interval (CI) 2.4-5.8], and the 5-year overall survival (OS) rate was 43% (95% CI 33-53). In multivariate analysis, liver primary site [relative risk (RR) = 12.62], performance status (PS) of two or more (RR = 3.83), presence of metastases at diagnosis (RR = 2.50), soft tissue tumor (RR = 0.31) were correlated to OS. PS, liver and soft tissue tumors were identified as independent prognostic factors for progression-free survival.
Angiosarcomas have an overall poor outcome, but with a clearly distinct prognosis depending on the primary site.
血管肉瘤罕见且具有异质性，本研究通过回顾性研究来描述其自然病程。
我们回顾了1980年至2004年在法国肉瘤研究组三个机构接受治疗的161例血管肉瘤患者的病历。分析了生存率及生存的预后因素。
中位年龄为52岁。原发部位为乳腺（35%）、皮肤（20%）和软组织（13%）。初诊时，31例（19%）已有转移。121例（75%）患者首先接受手术治疗，其中34例和32例分别联合化疗或放疗。这121例患者中有90例（74%）复发，大多为局部复发（50例）。自初诊起平均8.1年，123例（76%）患者病情进展，76例（47%）死亡。中位生存期为3.4年[95%置信区间（CI）2.4 - 5.8]，5年总生存率（OS）为43%（95% CI 33 - 53）。多因素分析显示，肝脏原发部位[相对危险度（RR）= 12.62]、体能状态（PS）为二级或更差（RR = 3.83）、诊断时存在转移（RR = 2.50）、软组织肿瘤（RR = 0.31）与总生存期相关。PS、肝脏和软组织肿瘤被确定为无进展生存期的独立预后因素。
血管肉瘤总体预后较差，但根据原发部位预后明显不同。
