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Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review.
- Source :
-
Journal of cardiothoracic surgery [J Cardiothorac Surg] 2024 May 14; Vol. 19 (1), pp. 288. Date of Electronic Publication: 2024 May 14. - Publication Year :
- 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.<br /> (© 2024. Crown.)
- Subjects :
- Humans
Male
Middle Aged
Diagnosis, Differential
Vasculitis diagnosis
Diagnostic Errors
Pulmonary Artery diagnostic imaging
Pulmonary Artery surgery
Pulmonary Artery pathology
Sarcoma diagnosis
Sarcoma surgery
Pulmonary Valve diagnostic imaging
Vascular Neoplasms diagnosis
Vascular Neoplasms surgery
Vascular Neoplasms diagnostic imaging
Subjects
Details
- Language :
- English
- ISSN :
- 1749-8090
- Volume :
- 19
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Journal of cardiothoracic surgery
- Publication Type :
- Academic Journal
- Accession number :
- 38745263
- Full Text :
- https://doi.org/10.1186/s13019-024-02700-3