Pulmonary valve stenosis due to undifferentiated pleomorphic sarcoma

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State: Public
Version: Final published version
Serval ID
serval:BIB_836C651E0085
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Pulmonary valve stenosis due to undifferentiated pleomorphic sarcoma
Journal
Cardiovascular Medicine
Author(s)
Antiochos P., Monney P., Qanadli SD., Schlueter L., Rotman S., Descloux E., Niclauss L., Hurni M., Muller O.
Publication state
Published
Issued date
06/2015
Volume
18
Number
6
Pages
186-190
Language
english
Abstract
Pulmonary artery sarcomas are uncommon intracardiac tumours with poor prognosis. We report the case of a 69-year-old woman in good health presenting with rapidly progressive dyspnoea and an unfamiliar systolic murmur. Echocardiography revealed pulmonary valve stenosis due to an obstructing mobile mass. Imaging studies confirmed the presence of a contrast-enhancing lesion adherent to the valve, extending into the pulmonary trunk and right ventricular outflow tract, and suggestive of malignancy.
Endovascular biopsy was attempted with no success. Surgical resection with autologous graft valve replacement and pulmonary artery reconstruction was performed. Postoperative histological examination
confirmed the diagnosis of an undifferentiated pleomorphic sarcoma. Pulmonary artery sarcoma should be considered as a rare differential diagnosis in patients presenting with dyspnoea and a crescendo-decrescendo systolic murmur increasing with inspiration. Echocardiography is a useful first diagnostic approach but multi-imaging assessment is almost always necessary for definite diagnosis. Our case provides insights into the challenges met by cardiologists, radiologists and cardiac surgeons in the management of such cases.
Create date
07/09/2015 10:58
Last modification date
20/08/2019 15:43
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