Objective: Pulmonary metastatic angiosarcoma is a rare, fatal disease that often presents as diffuse alveolar hemorrhage(DAH). In this report, clinical characteristics of pulmonary metastatic angiosarcoma were retrospectively reviewed. Methods: A total of 9 patients with angiosarcoma who presented as DAH were enrolled. Clinical data included age, gender, symptoms, smoking status, physical exam findings, pulmonary function tests, and radiology. Results: All patients were male with median age 41 years(range, 22 to 57 years). The most common symptom was hemoptysis(9/9). Other symptoms included dyspnea (5/9), cough(3/9), chest pain(3/9), fever(2/9,) and edema of the lower extremity and oliguria(4/9).The common misdiagnoses were tuberculosis(4/9), vasculitis(3/9) and other infection(1/9). Chest computed tomography showed bilateral,random distributed different-sized nodules(9/9),as well as ground-glass areas (9/9).The hearts, mainly right atrium, were the most common primary locations(7/9).Cardiac mass was the first manifestation in five patients by echocardiography(5/8).Two atrial masses were identified by computer tomography pulmonary angiography and magnetic resonance imaging respectively. Transbronchial lung biopsy failed to find malignancy. Computer tomography guided transthoracic needle biopsy was difficult to perform in most patients. Eight patients were diagnosed by surgical biopsy, either by lung biopsy(4/8) or cardiac biopsy(4/8).The median survival period was only 3 months after surgery. Conclusion: Metastatic pulmonary angiosarcoma should be considered in patients with DAH and multiple glass ground opacity and nodules on chest CT. Careful cardiologic monitoring is necessary. Surgical biopsy is reliable for diagnosis.
目的： 通过分析以弥漫性肺泡出血为首发表现的肺转移性血管肉瘤的临床资料，旨在提高临床医生对其的认识。 方法： 回顾性分析9例明确诊断为肺转移性血管肉瘤患者的临床资料和诊治过程。 结果： 9例肺转移性血管肉瘤患者均为男性，平均年龄41（22~57）岁，均以咯血（9例）为首发症状，可伴进行性加重的呼吸困难（5例）、咳嗽（3例）、胸痛（3例）、低热（2例），以及下肢水肿和少尿（4例），平均病程5.0（2.0~12.0）个月。误诊为肺结核4例，血管炎3例，非结核性感染性疾病1例。胸部CT表现为同时存在的双肺多发随机分布大小不等实性结节（9例）和多发磨玻璃影（9例），进展迅速。2例正电子发射计算机断层扫描（PET-CT）提示恶性疾病。超声心动图检查发现5例右心肿物。磁共振成像（MRI）发现1例、CT血管造影（CTA）发现1例右心房肿物。9例患者均为肺转移性血管肉瘤，原发部位为心脏7例，肝脏1例，不明1例。6例患者支气管镜检查见气道内新鲜血液，支气管肺泡灌洗液呈血性，无恶性证据。所有患者均经组织病理学确诊。1例行CT引导下经皮肺穿刺，4例行心脏肿物活检，4例行胸腔镜肺活检。8例患者平均总生存期为3个月（1例患者出院后失访）。 结论： 肺转移性血管肉瘤是一种罕见病，以弥漫性肺泡出血为首发症状较为少见，胸部CT上多发磨玻璃影和多发结节的同时存在可提示该病。对此类患者应仔细筛查心脏。手术活检是诊断的金标准。患者预后差，生存期短。.
Keywords: Diffuse alveolar hemorrhage; Hemangiosarcoma; Hemoptysis; Neoplasm, metastasis.