Division of Vascular and Endovascular Surgery, Mayo Clinic, Rochester, MN, USA.
J Vasc Surg. 2013 Mar;57(3):756-64. doi: 10.1016/j.jvs.2012.09.023. Epub 2013 Jan 9.
Primary angiosarcomas originating from the heart, aorta, or great vessels are extremely rare and hence poorly understood. We reviewed our experience to identify a preferred diagnostic and treatment strategy and evaluate the role of adjunctive therapy.
We reviewed the clinical data of all patients diagnosed with primary angiosarcoma of the heart, aorta, and great vessels from 1985 to 2011, including presentation, diagnosis, management, and outcomes.
Thirteen patients (five males and eight females; mean age, 54 ± 4 years) had primary angiosarcoma arising from the aorta (n = 7), heart (n = 3), pericardium (n = 2), and pulmonary artery (n = 1). Patients with aortic tumors most commonly presented with lower extremity claudication (n = 2), renovascular hypertension (n = 3), abdominal pain (n = 5), and weight loss (n = 4). Patients with cardiac and pericardial tumors presented with dyspnea (n = 5) due to pleural effusion or cardiac tamponade. All 13 patients underwent computed tomographic scan, which demonstrated irregular, lobulated mass/thrombus with peripheral enhancement, and eight patients underwent diagnostic echocardiography. Metastatic disease was present in 10 patients. The most common site was the lungs (n = 6). All except one patient exhibited high-grade morphology histopathologically. Nine patients were treated surgically: resection with aortic reconstruction (n = 5), thromboendarterectomy (n = 2), pericardiectomy/atrial septal resection with patch reconstruction (n = 2), and just biopsy (n = 1). Adjunctive treatment included chemotherapy (n = 6) and radiation (n = 4). Median survival was 8 months (range, 1-75 months). Patients treated with all three treatment modalities had longer survival than did patients treated with a single modality (P = .013). Patients treated with chemotherapy had a more favorable survival than did those without chemotherapy (P = .048).
Primary angiosarcoma of the heart and great vessels is rare but is a harbinger of poor prognosis. Pathologic examination is necessary to confirm the diagnosis. Combined therapy with surgical resection and chemoradiotherapy offers patients the best survival.
原发于心脏、主动脉或大血管的血管肉瘤极为罕见，因此了解甚少。我们回顾了经验，以确定首选的诊断和治疗策略，并评估辅助治疗的作用。
我们回顾了 1985 年至 2011 年间所有诊断为心脏、主动脉和大血管原发性血管肉瘤患者的临床资料，包括表现、诊断、治疗和结果。
13 例患者（男性 5 例，女性 8 例；平均年龄 54 ± 4 岁）患有源自主动脉（n = 7）、心脏（n = 3）、心包（n = 2）和肺动脉（n = 1）的原发性血管肉瘤。主动脉肿瘤患者最常见的表现为下肢跛行（n = 2）、肾血管性高血压（n = 3）、腹痛（n = 5）和体重减轻（n = 4）。心脏和心包肿瘤患者因胸腔积液或心脏压塞而出现呼吸困难（n = 5）。所有 13 例患者均行 CT 扫描，显示不规则、分叶状肿块/血栓，伴周围强化，8 例患者行超声心动图检查。10 例患者存在转移性疾病，最常见的部位是肺部（n = 6）。除 1 例外，所有患者的组织病理学均为高级别形态。9 例患者接受手术治疗：主动脉重建切除（n = 5）、血栓内膜切除术（n = 2）、心包切除术/房间隔切除术联合补片重建（n = 2）和单纯活检（n = 1）。辅助治疗包括化疗（n = 6）和放疗（n = 4）。中位生存时间为 8 个月（范围 1-75 个月）。接受三种治疗方式的患者比接受单一治疗方式的患者生存时间更长（P =.013）。接受化疗的患者比未接受化疗的患者生存更有利（P =.048）。
心脏和大血管的原发性血管肉瘤罕见，但预示预后不良。病理检查对明确诊断是必要的。手术切除联合放化疗是患者获得最佳生存的最佳选择。
