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JYMS : Journal of Yeungnam Medical Science

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Jung Min Ahn 1 Article
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
Yeungnam Univ J Med. 2017;34(2):279-284.   Published online December 31, 2017
DOI: https://doi.org/10.12701/yujm.2017.34.2.279
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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.

JYMS : Journal of Yeungnam Medical Science