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Heart, Lung and Circulation

Clinical Features and Outcomes of Pulmonary Artery Sarcoma

  • Author Footnotes
    1 These authors contributed equally to this work.
    Jifeng Li
    Footnotes
    1 These authors contributed equally to this work.
    Affiliations
    Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China
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  • Author Footnotes
    1 These authors contributed equally to this work.
    Lin Liu
    Footnotes
    1 These authors contributed equally to this work.
    Affiliations
    Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China
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  • Ling-Xie Song
    Affiliations
    Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Yu-Hui Zhang
    Affiliations
    Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China
    Search for articles by this author
  • Yan Liu
    Affiliations
    Department of Cardiac Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Song Gu
    Affiliations
    Department of Cardiac Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Jian-Feng Wang
    Affiliations
    Department of Interventional Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Qiang Huang
    Affiliations
    Department of Interventional Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Zhan-Hong Ma
    Affiliations
    Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Xiao-Juan Guo
    Affiliations
    Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Min-Fu Yang
    Affiliations
    Department of Nuclear Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Wei Jiang
    Affiliations
    Department of Echocardiography, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Feng Li
    Affiliations
    Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
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  • Yuan-Hua Yang
    Correspondence
    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 100020
    Affiliations
    Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China

    Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China
    Search for articles by this author
  • Author Footnotes
    1 These authors contributed equally to this work.
Published:August 17, 2021DOI:https://doi.org/10.1016/j.hlc.2021.06.448

      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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