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Heart, Lung and Circulation

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Clinical Features and Outcomes of Pulmonary Artery Sarcoma - 10/01/22

Doi : 10.1016/j.hlc.2021.06.448 
Jifeng Li, MD, PhD a, b, c, d, 1 , Lin Liu, BS a, b, c, d, 1, Ling-Xie Song, MD, PhD e, Yu-Hui Zhang, MD a, b, c, d, Yan Liu, MD f, Song Gu, MD f, Jian-Feng Wang, MD g, Qiang Huang, MD g, Zhan-Hong Ma, MD h, Xiao-Juan Guo, MD h, Min-Fu Yang, MD i, Wei Jiang, MD j, Feng Li, MD, PhD e, Yuan-Hua Yang, MD, PhD a, b, c, d, ⁎
a Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
b Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
c Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
d Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China 
e Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
f Department of Cardiac Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
g Department of Interventional Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
h Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
i Department of Nuclear Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 
j Department of Echocardiography, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China 

∗Corresponding author at: Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, P. R. China 100020Department of Respiratory and Critical Care MedicineBeijing Chao-Yang HospitalCapital Medical University8 Gongren Tiyuchang NanluChaoyang DistrictBeijing100020P. R. China

Abstract

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.

Le texte complet de cet article est disponible en PDF.

Keywords : Pulmonary artery sarcoma, Pulmonary thromboembolism, Pulmonary endarterectomy, Outcome, Survival


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© 2021  Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Publié par Elsevier Masson SAS. Tous droits réservés.
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Vol 31 - N° 2

P. 230-238 - février 2022 Retour au numéro
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