Pulmonary Artery Sarcoma: Value of Dual-Energy CT (DECT)-Based Iodine Quantification on Multimodality Workup

A healthy 74-year-old man presented with increasing dyspnea for the last 5-months. Laboratory tests revealed thrombocytopenia (<60,000 platelets/μl) and increased D-dimer (1800 ng/mL).

Initial transthoracic echocardiography demonstrated elevated pulmonary artery systolic pressure (PASP = 54 mmHg) suggesting severe pulmonary hypertension. Dual-energy CT (DECT) pulmonary angiography was performed to rule out pulmonary thromboembolism (PTE). CT images showed a large low-attenuation filling defect occupying almost completely the luminal diameter of the pulmonary trunk and main pulmonary arteries (PA) that expanded the arterial wall (Fig. 1 Panels A–C).

With the clinical suspicion of pulmonary artery sarcoma (PAS) a cardiac-MRI (Panel E) was performed for better characterization. It showed subtle gadolinium enhancement suggesting a tumoral etiology rather than thrombus. The mass was surgically resected. Histological and immunohistochemical analysis revealed malignant cell infiltration (positive for MDM-2-gen, vimentin; negative for CD-34), and confirmed the diagnosis of PAS.

CITATION  Arch Bronconeumol. 2022 Jul;58(7):565.  doi: 10.1016/j.arbres.2021.08.017.  Epub 2021 Sep 3.