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Lijuan Qian, Jie Yu, Rosai-Dorfman disease of the ascending aorta and pulmonary artery mimicking intramural hematoma and mass, European Heart Journal, 2025;, ehaf250, https://doi.org/10.1093/eurheartj/ehaf250
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Extract
A 61-year-old man presented with chest tightness and dyspnoea for 6 months, along with elevated NT-proBNP levels (2110 pg/mL). Non-contrast CT (Panel A) revealed circumferential thickening of the ascending aortic wall, with a CT value of 65HU and inward displacement of local calcified plaque (white arrow), suggestive of an intramural hematoma. MRI showed circumferential thickening with slightly increased T1 and low T2 signals, and a small mass in the pulmonary trunk with isointense T1 and T2 signals (Panels B and C, white arrow). DWI shows restricted diffusion with high signal in the lesion (white arrow) (Panel D), and low signal on ADC (Panel E), while contrast-enhanced MRI revealed heterogeneous enhancement of the lesions (white arrow) (Panel F). MRPA identified a 25 × 24 mm filling defect in the pulmonary trunk, causing severe stenosis and partially extending to the superior wall (Panel G). Postoperatively, CTA showed near-complete resection of the pulmonary trunk mass with residual nodules (Panel I). Histopathology revealed proliferative tissue cells with infiltrated plasma cells and lymphocytes (hematoxylin-eosin stain, Panels J and K).