24例原发性血管肉瘤的临床病理特点及疗效分析

Clinical pathological characteristics and curative effect of primary angiosarcoma:a report of 24 cases

目的探讨血管肉瘤(AS)的临床病理特点及预后。方法回顾性分析1998年1月至2015年12月昆明医科大学第三附属医院收治的24例AS患者的临床及随访资料,其中单纯手术治疗8例,综合治疗(手术+化疗、手术+放射治疗、手术+放射治疗+化疗、手术+放射治疗+免疫治疗)16例。分析AS的临床特点及预后,采用Cox风险比例回归模型分析影响预后的因素。结果全组患者的中位无病生存期为12.0个月,其中局部复发9例(37.5%),远处转移18例(75.0%);中位生存期为24.0个月,1、3、5及10年生存率依次为65.2%、45.6%、30.4%及13.2%。单因素分析显示原发肿瘤大小、临床分期、恶性程度及不同治疗模式与预后有关。多因素分析提示原发肿瘤大小、恶性程度、临床分期及治疗模式均为独立预后因素。结论 AS的治疗应在根治性手术的基础上,辅以放化疗及靶向治疗等综合治疗。预后应综合考虑年龄、原发肿瘤大小、恶性程度、分期、治疗方法及复发转移时间等因素,但首次治疗采取综合治疗是关键。

Objective To explore the clinieopathological characteristics and prognosis of angiosarcoma （AS）. Methods In a retrospective analysis, the clinical and follow-up data of 24 patients with AS treated by the Third Affiliated Hospital of Kunming Medi- cal University from January 1998 to December 2015 were analyzed. Eight cases only received the surgery and other 16 cases received comprehensive treatment, including surgery plus chemotherapy, surgery plus radiotherapy, surgery plus radiotherapy and chemotherapy and surgery plus radiotherapy and immunotherapy. The clinical characteristics and prognosis of AS were analyzed. The factors influen- cing the prognosis of the patients were analyzed with Cox risk proportional regression model. Results The medium disease-free survival of whole group was 12.0 months, including 9 cases （ 37.5% ） of local recurrence and 18 cases （ 75.0% ） of distant metastasis. The medium overall survival of whole group was 24. 0 months, and 1, 3, 5 and 10-year survival rates were 65.2%, 45.6%, 30.4% and 13.2%, respectively. Univariate analysis showed that tumor size, stage, grade malignancy and treatment patterns associated with prog- nosis. Multiple factors analysis showed tumor size, grade malignancy, stage and treatment patterns were independent prognostic factors. Conclusion The treatment of AS should adopt comprehensive therapy that based on the radical surgery and supplemented by radio- therapy, chemotherapy and targeted therapy, etc. Prognosis should consider synthetically age, tumor size, malignant degree, stage, treatment and relapse transfer. It is the key to take comprehensive treatment for the first time.