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Open Access 14-05-2024 | Sarcoma | Case Report

Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review

Authors: Harry James Smith, Fadi Al-Zubaidi, Daniel Sitaranjan, Steven Chiu, David Jenkins

Published in: Journal of Cardiothoracic Surgery | Issue 1/2024

Abstract

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

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Title: Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review
Authors: Harry James Smith
Fadi Al-Zubaidi
Daniel Sitaranjan
Steven Chiu
David Jenkins
Publication date: 14-05-2024
Publisher: BioMed Central
Keywords: Sarcoma
Vasculitis
Pulmonary Valve Replacement
Pulmonary Valve Replacement
Pulmonary Valve Replacement
Thoracic Tumor
Vasculitis
Published in: Journal of Cardiothoracic Surgery / Issue 1/2024
Electronic ISSN: 1749-8090
DOI: https://doi.org/10.1186/s13019-024-02700-3

ERS Congress 2024

Springer Medicine

Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review

Abstract

ERS Congress 2024

Springer Medicine

Abstract

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