作者：康世威,宫小薇,何平,崔江华,袁雅冬

单位：

050000 河北省石家庄市，河北医科大学第二医院呼吸与危重症医学二科通信作者：袁雅冬，E-mail：yuanyd1108@163.com

Units：

Department of Respiratory and Critical Care Medicine, the Second Hospital of Hebei Medical University, Shijiazhuang 050000,ChinaCorresponding author: YUAN Yadong, E-mail: yuanyd1108@163.com

关键词：

肿瘤; 原发性肺动脉肉瘤; 肺栓塞; 咯血; 超声支气管镜; 诊断;

Keywords：

Neoplasms; Primary pulmonary artery sarcoma; Pulmonary embolism; Hemoptysis; Endobronchialultrasound; Diagnosis

CLC：

DOI：

10.12114/j.issn.1008-5971.2021.00.225

Funds：

河北省医学科学研究重点课题计划（20160127）

摘要：

原发性肺动脉肉瘤(PPAS)是一种罕见的血管源性恶性肿瘤,病情进展迅速、诊断困难、预后极差。PPAS患者多以胸闷、胸痛、咯血或进行性呼吸困难为主要临床表现,甚至晕厥,临床极易误诊为肺血栓栓塞症(PTE),而早期诊断、肿瘤完整切除及合理的辅助治疗是延长PPAS患者生存时间的关键。本文报道了1例以咯血为主要临床表现的PPAS并PTE患者的诊疗经过,以提高临床医师对该病的诊疗水平。

Abstract：

【Abstract】 Primary pulmonary artery sarcoma (PPAS) is a rare vascular malignancy with rapid disease progression,difficult diagnosis and poor prognosis. PPAS is mostly characterized by chest tightness, chest pain, hemoptysis or progressivedyspnea, even accompanied by syncope, which is easily misdiagnosed as pulmonary thromboembolism (PTE) in clinical practice.Early diagnosis, complete tumor resection and reasonable adjuvant therapy are the keys to prolong the survival time of PPASpatients. In this paper, we reported the diagnosis and treatment of a patient with PPAS complicated with PTE with hemoptysis asthe main manifestation, in order to improve clinicians' diagnosis and treatment of the disease.

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