原发性血管肉瘤的临床特征和预后分析

Characteristics and outcomes of primary angiosarcoma

目的分析原发性血管肉瘤的临床特征、治疗情况和预后。方法回顾性分析1990年1月至2017年6月在北京协和医院收治的68例病理确诊为血管肉瘤患者的临床、手术病理资料和治疗情况,应用Kaplan-Meier法和Log rank检验进行单因素生存分析,采用Cox回归模型进行多因素生存分析。结果68例患者中,男38例,女30例;确诊时中位年龄50.5岁,从症状发生到确诊的时间为(7.5±7.5)个月,发病部位包括头面部、乳腺、胸壁、肺、心脏、肝脏、脾脏、四肢和骨骼等。确诊时肿瘤大小为(7.4±7.3)cm,局限性肿瘤(Ⅰ+Ⅱ期)28例,转移性肿瘤(Ⅲ+Ⅳ期)40例。单纯手术37例;单纯放疗3例;单纯化疗5例;综合治疗16例,包括手术+放疗5例,手术+化疗3例,手术+介入治疗4例,放疗+化疗2例,放疗+介入治疗1例,手术+放疗+化疗+靶向治疗1例;5例患者仅接受姑息对症治疗;2例确诊后失访。50例患者获得随访,中位生存时间为8.5个月。转移性血管肉瘤患者的中位生存时间为6.6个月,明显短于局限性血管肉瘤患者(15.0个月,P=0.020);心脏血管肉瘤患者的中位生存时间为3.0个月,明显短于其他部位血管肉瘤患者(11.5个月,P=0.010);接受综合治疗患者的中位生存时间为31.0个月,明显长于非综合治疗的患者(5.6个月,P=0.007)。多因素分析也显示,分期、是否发生于心脏和是否接受综合治疗均为影响原发性血管肉瘤患者预后的独立因素(均P<0.05)。结论血管肉瘤是罕见的恶性肿瘤,心脏血管肉瘤和转移性血管肉瘤的预后较差,综合治疗可改善血管肉瘤患者的预后。

Objective The study was designed to analyze the clinicopanthologic characteristics, treatments and outcomes of a series of patients with primary angiosarcoma. Methods The clinical, surgical and pathological data and treatment of 68 patients with pathologically confirmed angiosarcoma admitted to Peking Union Medical College Hospital from January 1990 to June 2017 was retrospectively analyzed. Kaplan-Meier method and Log rank test were used for univariate survival analysis and Cox regression model was used for multivariate survival analysis. Results A total of 68 patients were enrolled, 38 were male, 30 were female. The median age at diagnosis was 50.5 years. The time from symptom onset to diagnosis was (7.5±7.5) months. The primary sites included face and scalp, breast, chest wall, lung, heart, liver, spleen, extremities, bones and so on. At diagnosis, the mean size of tumors were (7.4±7.3) cm, 28 patients (41.2%) had localized disease (stage Ⅰ+Ⅱ) and 40 patients had metastatic disease (stage Ⅲ+Ⅳ). There were 37 patients treated with surgery alone, three receiving radiotherapy alone, five receiving chemotherapy alone and sixteen receiving comprehensive treatment with 5 underwent surgery plus radiotherapy, three treated by surgery plus chemotherapy, four had surgery plus interventional therapy, two had chemoradiotherapy, one had radiotherapy and interventional therapy and 1 had surgery plus chemoradiotherapy and targeted therapy. Five patients received only palliative treatment, and 2 patients lost follow-up after diagnosed. Fifty patients were followed up with a median overall survival time of 8.5 months. The median survival time of patients with metastatic angiosarcoma was 6.6 months, significantly shorter than that of patients with localized disease (15.0 months, P=0.020). The median survival time of patients with cardiac angiosarcoma was 3.0 months, significantly shorter than that of patients with angiosarcoma at other sites (11.5 months, P=0.010). The median survival time of patients receiving comprehensive treatment was 31.0 months, significantly longer than that of patients without comprehensive treatment (5.6 months, P=0.007). Multivariate analysis showed that staging, heart occurrence and comprehensive treatment were independent factors for the prognosis of primary angiosarcoma (all P<0.05). Conclusions Angiosarcoma is a rare malignancy, and patients with metastatic disease or cardiac occurence have poor prognosis. Comprehensive treatment can improve the prognosis of patients with angiosarcoma.