Adeli Hassan, Nemati Bardia, Jandaghi Mahboubeh, Riahi Mohammad Mahdi, Salarvand Fatemeh
Clinical Research Development Center, Qom University of Medical Sciences, Qom, Iran.
General Practitioner, Clinical Research Development Center, Qom University of Medical Sciences, Qom, Iran.
ARYA Atheroscler. 2014 Mar;10(2):133-6.
Primary pulmonary artery sarcomas are very rare and their histologic type called leiomyosarcoma is even rarer. These tumors are frequently misdiagnosed as pulmonary thromboembolism in clinical settings. Many patients receive anticoagulant therapy without response, and many are diagnosed postmortem only. Most of the tumors reported in the literature have involved the right ventricular outflow tract and the main pulmonary trunk, often extending into the main pulmonary artery (MPA) branches.
A 64-year-old woman presented with weakness, fatigue, malaise, dyspnea, and marked elevation of pulmonary artery pressure was admitted to our hospital. She was initially diagnosed with chronic pulmonary thromboembolism, and chest computed tomography (CT) scan revealed lobulated heterogeneous left hilar mass extended to precarinal and subcarinal space. Magnetic resonance imaging (MRI) demonstrated a polypoid lesion at the trunk with extension to left MPA and its first branch. The patient was operated, and a yellowish-shiny solid mass in pulmonary trunk was seen intra-operation and pulmonary endarterectomy was performed. Her tumor was pathologically diagnosed as pulmonary artery leiomyosarcoma.
Clinicians must consider pulmonary artery sarcoma when making the differential diagnosis for patients with pulmonary artery masses. The clinical prediction scores and the CT and MRI findings can help identifying patients with pulmonary artery sarcoma.
原发性肺动脉肉瘤非常罕见，其组织学类型为平滑肌肉瘤则更为罕见。这些肿瘤在临床中常被误诊为肺血栓栓塞症。许多患者接受抗凝治疗无效，且许多患者仅在尸检时才被诊断出来。文献报道的大多数肿瘤累及右心室流出道和主肺动脉干，常延伸至主肺动脉（MPA）分支。
一名64岁女性因虚弱、疲劳、不适、呼吸困难及肺动脉压显著升高入院。她最初被诊断为慢性肺血栓栓塞症，胸部计算机断层扫描（CT）显示分叶状不均匀的左肺门肿块延伸至气管隆突前和隆突下间隙。磁共振成像（MRI）显示主干有一息肉样病变，延伸至左主肺动脉及其第一分支。患者接受了手术，术中可见肺动脉干内有一淡黄色有光泽的实性肿块，并进行了肺动脉内膜剥脱术。她的肿瘤经病理诊断为肺动脉平滑肌肉瘤。
临床医生在对肺动脉肿块患者进行鉴别诊断时必须考虑肺动脉肉瘤。临床预测评分以及CT和MRI表现有助于识别肺动脉肉瘤患者。
