Pulmonary Artery Sarcoma Diagnosed by Transbronchial Endosonographic (EBUS-TBNA) Approach

Background: Pulmonary Artery Sarcomas are the rarest and the most aggressive vascular tumors. Their accurate diagnosis is challenging due to the lack of specific symptoms and clinical manifestations and they are often initially misdiagnosed and managed as chronic pulmonary thromboembolic disease until an alternate diagnosis is suspected based on the lack of response to anticoagulants. Proper management is thus often delayed until histological confirmation. Moreover, the lack of consensus regarding their diagnosis and treatment further contributes to their reported high mortality [1]. Case Presentation: We present the case of a 34-year-old male, nonsmoker, who presented with hemoptysis, fatigue and dyspnea on exertion 3 months prior to admission. His chest x-ray showed enlargement of the left hilum and chest CT revealed a large intraluminal low attenuation filling defect at the level of the left PA. Chest MRI confirmed the presence of a large endovascular tumor of the left PA. Before referring the patient for surgical biopsy, he underwent bronchoscopy during which the vascular tumor was successfully located, observed and sampled by endobronchial ultrasound (EBUS) transbronchial needle aspiration (TBNA). Tissue biopsy through EBUS-TBNA revealed an intimal sarcoma of the PA. The patient was subsequently referred for surgical excision with curative intent. Conclusion: Bronchoscopy and EBUS-TBNA may safely and accurately diagnose even rare pathologies of the mediastinal vasculature adjacent to the airways through transbronchial approach.