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Yeungnam Univ J Med > Volume 34(2); 2017 > Article
Yeungnam University Journal of Medicine 2017;34(2):279-284.
DOI: https://doi.org/10.12701/yujm.2017.34.2.279    Published online December 31, 2017.
Pulmonary artery sarcoma manifesting as a main pulmonary artery stenosis diagnosed by 18F-FDG PET/CT
Hoonhee Lee, Han Bit Park, Yun Kyung Cho, Jung Min Ahn, Sang Min Lee, Jae Seung Lee, Dae Hee Kim
1Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
2Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. jsdoc1186@daum.net
3Pulmonary Hypertension and Venous Thrombosis Center, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
4Department of Cardiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
5Department of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Abstract
Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.
Key Words: Pulmonary artery, Stenosis, Sarcoma, Positron emission tomography
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