Extranodal Rosai-Dorfman disease involving the pulmonary artery.

A 61-year-old woman underwent computed tomography (CT) angiography for evaluation of syncope and was found to have a central pulmonary artery filling defect (Fig. 1). During an attempted intravascular ultrasound-guided biopsy of the lesion, the patient coded and died. Autopsy confirmed the case as a rare presentation of Rosai-Dorfman disease (Fig. 2 and Supplementary Figs 1 and 2). Supplementary material is available at EJCTS online. Figure 1: CT angiogram (an axial view at the level of carina) showing a large filling defect within the main pulmonary artery. Figure 2: An explanted heart–lung block (a dorsal view with pulmonary trunk cut open) revealing a 6.4 × 3.2 × 3.0 cm, tan, well-circumscribed lesion with the pulmonary trunk.