Medalie N S, Vallejo C E, Wasserman P
Department of Pathology, Long Island Jewish Medical Center, New Hyde Park, New York 11040, USA.
Acta Cytol. 1998 Jul-Aug;42(4):968-72. doi: 10.1159/000331978.
Primary pulmonary artery sarcomas (PPASs) are uncommon neoplasms with a poor prognosis. They often present with symptoms of pulmonary thromboembolus. They may be locally aggressive and give rise to embolic pulmonary metastases. Extrathoracic metastases are uncommon. They are usually diagnosed late in the course of the disease or at autopsy. Radiographic studies alone are insufficient to definitively diagnose PPAS.
A 68-year-old female presented with symptoms consistent with pulmonary thromboemboli. Computed tomography of the chest with intravenous contrast revealed an intraluminal filling defect involving the left main pulmonary artery. Despite anticoagulation therapy, the symptoms persisted. Four months after the initial presentation, the patient developed multiple nodules in the left lung. Fine needle aspiration (FNA) of one of the nodules showed loosely cohesive clusters of pleomorphic and spindle-shaped malignant cells and isolated ones. These cells were embedded in extracellular, metachromatic material. They were vimentin positive and keratin negative. There was no evidence of extrapulmonary disease. A diagnosis of metastatic pulmonary artery sarcoma was made and confirmed on histology. Three weeks following the pneumonectomy, the patient died of postoperative complications.
Patients with PPAS often present with symptoms of atypical or nonresolving pulmonary thromboemboli. The interval between presentation and definitive diagnosis is usually delayed and probably affects the prognosis. The timely use of FNA techniques could result in an earlier diagnosis, possibly improving outcome.
原发性肺动脉肉瘤（PPASs）是一种罕见的肿瘤，预后较差。它们常表现为肺血栓栓塞的症状。它们可能具有局部侵袭性，并可导致栓塞性肺转移。胸外转移并不常见。它们通常在疾病晚期或尸检时被诊断出来。仅靠影像学检查不足以明确诊断PPAS。
一名68岁女性出现与肺血栓栓塞相符的症状。胸部增强计算机断层扫描显示左主肺动脉腔内充盈缺损。尽管进行了抗凝治疗，症状仍持续存在。初次就诊四个月后，患者左肺出现多个结节。对其中一个结节进行细针穿刺抽吸（FNA），结果显示为多形性和梭形恶性细胞的松散聚集簇以及单个细胞。这些细胞包埋在细胞外异染性物质中。它们波形蛋白阳性，角蛋白阴性。没有肺外疾病的证据。诊断为转移性肺动脉肉瘤，并经组织学证实。肺切除术后三周，患者死于术后并发症。
PPAS患者常表现为非典型或无法缓解的肺血栓栓塞症状。从出现症状到明确诊断的间隔通常会延迟，这可能会影响预后。及时使用FNA技术可能会更早诊断，有可能改善预后。
