Abstract：
UNASSIGNED: Pulmonary artery sarcomas (PAS) are rare tumours causing an insidiously progressive obstruction of the pulmonary circulation. The clinical presentation is often indistinguishable from chronic thromboembolic pulmonary hypertension (CTEPH). However, the atypical appearance of a heterogeneous filling defect in CT pulmonary angiography (CTPA) should prompt further investigation.
UNASSIGNED: A previously healthy young man presented with massive haemoptysis, acute respiratory distress, and progressive exertional dyspnea since the year before. Echocardiography demonstrated severe right ventricular dysfunction and highly probable pulmonary hypertension. CTPA revealed an extensive filling defect with an appearance concerning PAS. Due to syncopal episodes at rest, the patient underwent urgent pulmonary artery endarterectomy (PEA). A massive tree-like tumour was excised as a result. Post-operatively, reperfusion injury and refractory pulmonary oedema mandated extracorporeal membrane oxygenation (ECMO). Unfortunately, ECMO was complicated with massive haemolysis and acute kidney injury. The patient succumbed to multi-organ failure. Through tissue analysis established a diagnosis of embryonal rhabdomyosarcoma.
UNASSIGNED: Unfortunately, the patient had not reached out for his worsening dyspnea. PASs should not be mistaken for a thrombus and anticoagulation should be avoided. The urgent condition precluded biopsy and tissue diagnosis. Similarly, neoadjuvant chemotherapy was not feasible. Post-operatively, reperfusion injury and pulmonary oedema ensued, which mandated ECMO. This complication should be anticipated preoperatively. There is a need for more data on PASs to establish a consensus for management.
UNASSIGNED: A previously healthy young man presented with massive haemoptysis, acute respiratory distress, and progressive exertional dyspnea since the year before. Echocardiography demonstrated severe right ventricular dysfunction and highly probable pulmonary hypertension. CTPA revealed an extensive filling defect with an appearance concerning PAS. Due to syncopal episodes at rest, the patient underwent urgent pulmonary artery endarterectomy (PEA). A massive tree-like tumour was excised as a result. Post-operatively, reperfusion injury and refractory pulmonary oedema mandated extracorporeal membrane oxygenation (ECMO). Unfortunately, ECMO was complicated with massive haemolysis and acute kidney injury. The patient succumbed to multi-organ failure. Through tissue analysis established a diagnosis of embryonal rhabdomyosarcoma.
UNASSIGNED: Unfortunately, the patient had not reached out for his worsening dyspnea. PASs should not be mistaken for a thrombus and anticoagulation should be avoided. The urgent condition precluded biopsy and tissue diagnosis. Similarly, neoadjuvant chemotherapy was not feasible. Post-operatively, reperfusion injury and pulmonary oedema ensued, which mandated ECMO. This complication should be anticipated preoperatively. There is a need for more data on PASs to establish a consensus for management.
摘要：
背景：肺动脉肉瘤（PAS）是一种罕见的肿瘤，可导致肺循环潜在的进行性阻塞。临床表现通常与慢性血栓栓塞性肺动脉高压（CTEPH）无法区分。然而,CT肺动脉造影(CTPA)中不均匀充盈缺损的不典型表现应提示进一步研究.
方法：一个以前健康的年轻人出现大咯血，急性呼吸窘迫,从前一年开始进行性劳力性呼吸困难。超声心动图显示严重的右心室功能障碍和极可能的肺动脉高压。CTPA显示出广泛的填充缺陷，外观与PAS有关。由于休息时的晕厥发作，患者接受了紧急肺动脉内膜切除术(PEA).结果切除了一个巨大的树状肿瘤。术后,再灌注损伤和难治性肺水肿强制体外膜氧合（ECMO）。不幸的是,ECMO并发大量溶血和急性肾损伤。患者死于多器官衰竭。通过组织分析确立了胚胎性横纹肌肉瘤的诊断价值。
结论：不幸的是，患者没有因呼吸困难恶化而伸出援手。PASs不应被误认为是血栓，应避免抗凝。紧急情况排除了活检和组织诊断。同样，新辅助化疗不可行.术后,再灌注损伤和肺水肿随之而来，它授权ECMO。这种并发症应在术前预料到。需要更多有关PAS的数据，以建立管理共识。
方法：一个以前健康的年轻人出现大咯血，急性呼吸窘迫,从前一年开始进行性劳力性呼吸困难。超声心动图显示严重的右心室功能障碍和极可能的肺动脉高压。CTPA显示出广泛的填充缺陷，外观与PAS有关。由于休息时的晕厥发作，患者接受了紧急肺动脉内膜切除术(PEA).结果切除了一个巨大的树状肿瘤。术后,再灌注损伤和难治性肺水肿强制体外膜氧合（ECMO）。不幸的是,ECMO并发大量溶血和急性肾损伤。患者死于多器官衰竭。通过组织分析确立了胚胎性横纹肌肉瘤的诊断价值。
结论：不幸的是，患者没有因呼吸困难恶化而伸出援手。PASs不应被误认为是血栓，应避免抗凝。紧急情况排除了活检和组织诊断。同样，新辅助化疗不可行.术后,再灌注损伤和肺水肿随之而来，它授权ECMO。这种并发症应在术前预料到。需要更多有关PAS的数据，以建立管理共识。
