Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 6, 2021; 9(16): 3960-3965
Published online Jun 6, 2021. doi: 10.12998/wjcc.v9.i16.3960
Undifferentiated intimal sarcoma of the pulmonary artery: A case report
Xin Li, Liu Hong, Xiao-Yan Huo
Xin Li, Liu Hong, Xiao-Yan Huo, Department of Ultrasonography, Chinese People's Liberation Army General Hospital-Sixth Medical Center, Beijing 100048, China
Author contributions: Li X and Huo XY contributed to the echocardiographic consultation and manuscript writing and revision; Liu H contributed to the pathological analysis and literature research; all authors read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xin Li, PhD, Department of Ultrasonography, Chinese People's Liberation Army General Hospital-Sixth Medical Center, No. 6 Fucheng Road, Haidian District, Beijing 100048, China. lixin_dp@163.com
Received: December 2, 2020
Peer-review started: December 2, 2020
First decision: January 29, 2021
Revised: February 16, 2021
Accepted: March 29, 2021
Article in press: March 29, 2021
Published online: June 6, 2021
Abstract
BACKGROUND

Since 1923, only a few hundred cases of pulmonary arterial sarcoma (PAS) have been reported. It is easy for PAS to be misdiagnosed as pulmonary thromboembolism, which makes treatment difficult. The median survival time without surgical treatment for PAS is only 1.5-3 mo. Echocardiography is widely used in screening for pulmonary artery space-occupying lesions in patients with chest pain, dyspnea, and cough; furthermore, it is typically considered the first imaging examination for patients with PAS.

CASE SUMMARY

In May 2017, a 39-year-old male patient experienced chest pain with no particular obvious cause. At that time, the cause was thought to be pulmonary embolism. In July 2017, positron emission tomography–computed tomography revealed space-occupying lesions in the right lung and multiple metastases in both lungs. The lesions of the right lung were biopsied, and pathology revealed undifferentiated sarcoma. Chemotherapy had been performed since July 2017 in another hospital. In December 2019, the patient was admitted to our hospital for the sake of CyberKnife treatment. Echocardiography suggested: (1) A right ventricular outflow tract (RVOT) solid mass of the main pulmonary artery; and (2) mild pulmonary valve regurgitation. Ultrasonography showed the absence of a thrombus in the deep veins of either lower limb.

CONCLUSION

PAS is a single, central space-occupying lesion involving the RVOT and pulmonary valve. Echocardiography of PAS has its own characteristics.

Keywords: Echocardiography, Computed tomography, Pulmonary arterial sarcoma, Pulmonary thromboembolism, Main pulmonary artery, Right ventricular outflow tract, Case report

Core Tip: Echocardiography has a high sensitivity in the diagnosis of pulmonary artery space-occupying lesions. The misdiagnosis of pulmonary arterial sarcoma (PAS) by echocardiography is attributed to insufficient knowledge of this disease. If the following four features are detected, a diagnosis of PAS should be considered: (1) A single pulmonary arterial mass involving the right ventricular outflow tract or pulmonary valve; (2) varying degrees of pulmonary stenosis detected based on ultrasonic measurements; (3) the mass can move slightly and blood supply can be seen inside; and (4) use of ultrasonography of the lower extremity and inferior vena cava to exclude thrombus.