Objectives
A retrospective cohort study was designed to describe the clinical features and outcomes
of pulmonary artery sarcoma (PAS).
Methods
Twenty-two (22) consecutive patients diagnosed with PAS by pathological examination
were enrolled and followed up until they died or until January 2020. The medical records
were retrospectively reviewed to evaluate the clinical characteristics, image findings,
and outcomes.
Results
1) Twenty-one (21, 95.5%) patients were firstly misdiagnosed. Dyspnoea was the most
common presenting symptom (19 of 22, 86.4%). 2) Filling defects in the right pulmonary
artery were seen in 17 patients (77.3%) with computed tomography pulmonary angiography
or magnetic resonance pulmonary angiography. Among those patients, 14 underwent positron
emission tomography-computed tomography detection and 13 (92.9%) were found to have
increased uptake value in the pulmonary artery. 3) The median survival (from diagnosis
to death or January 2020) of the total series was 11.6 months (range, 0.7–68.5 months).
The estimated cumulative survival rates at 1, 2, and 3 years were 52.6%, 32.8%, and
19.7%, respectively. Patients who received surgery and/or chemo-radiotherapy treatment
had a better survival rate compared with patients without treatment (the estimated
cumulative survival rates at 1, 2, and 3 years were 60.3%, 39.1%, and 29.3%, respectively,
vs 33.3%, 16.6%, and 0, accordingly) and better survival time (median survival 17.02
vs 3.16 months, respectively) (p=0.025).
Conclusions
Pulmonary artery sarcoma is easily misdiagnosed, as the symptoms and routine image
detection are nonspecific. Positron emission tomography-computed tomography may be
helpful in diagnosis. Surgery and/or chemo-radiotherapy offer a chance for better
outcomes.
Keywords
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Article info
Publication history
Published online: August 17, 2021
Accepted:
June 8,
2021
Received in revised form:
May 28,
2021
Received:
October 5,
2020
Identification
Copyright
© 2021 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
