<abstract xmlns="http://www.w3.org/1999/xhtml">
<sec><h3>Background: </h3>
<p>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.</p>
</sec>
<sec><h3>Case Presentation: </h3>
<p>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.</p>
</sec>
<sec><h3>Discussion:</h3>
<p>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.</p>
</sec>
</abstract>

Background: 
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.

Case Presentation: 
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.

Discussion:
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.

Massive primary pulmonary artery rhabomyosarcoma: A case report

Tuberculosis and Lung Diseases Research Center

Department of Anesthesiology, Faculty of Medicine

Romanian Journal of Internal Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"Massive primary pulmonary artery rhabomyosarcoma: A case report"}

Background: 
Pulmonary artery sarcomas (PAS) are rare tumours causing an insidiously progressive obstruction of the pulmonary circulation. The clinical...

Massive primary pulmonary artery rhabomyosarcoma: A case report

Published Online: Mar 23, 2024

Page range: 67 - 74

Received: May 31, 2023

DOI: https://doi.org/10.2478/rjim-2023-0032

Keywords
Pulmonary Hypertension, Pulmonary Artery, Rhabdomyosarcoma, Endarterectomy, Computed Tomography Angiography

© 2024 Farid Rashidi et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Massive primary pulmonary artery rhabomyosarcoma: A case report

Published Online: Mar 23, 2024

Page range: 67 - 74

Received: May 31, 2023

DOI: https://doi.org/10.2478/rjim-2023-0032

KeywordsPulmonary Hypertension, Pulmonary Artery, Rhabdomyosarcoma, Endarterectomy, Computed Tomography Angiography

© 2024 Farid Rashidi et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Keywords
Pulmonary Hypertension, Pulmonary Artery, Rhabdomyosarcoma, Endarterectomy, Computed Tomography Angiography