Spindle-cell sarcoma involving the major pulmonary arteries

Abstract

Primary pulmonary vasculature tumors are exceptionally rare, with only a few reported cases. Signs and symptoms of such neoplasms vary but include dyspnea, cough, and chest pain. This condition is associated with a high mortality rate and is easily misdiagnosed as a pulmonary artery embolism. We pre-sent a case of pulmonary artery sarcoma that demonstrates the value of cardiac magnetic resonance imaging for accurate diagnosis.

Figure 1.

(a, b) Contrast-enhanced CT image at the first admission demonstrates central complete occlusion of the right pulmonary artery (PA) by a hypodense mass exhibiting polypoid extension along the superior wall of the main pulmonary artery (black arrow) and the left pulmonary artery (white arrow). (c, d) Follow-up imaging 18 days later shows essentially no change of the occluding mass despite anticoagulant treatment.

Figure 2.

Cardiac magnetic resonance imaging left ventricular outflow tract views. (a) Gradient-recalled echo demonstrates a mass infiltrating the wall of the main pulmonary artery. (b, c) Double inversion turbo spin echo imaging demonstrates a heterogeneous signal intensity pattern of the mass using both (b) T1-weighted and (c) T2-weighted images. (d) Inversion recovery delayed enhancement demonstrates heterogeneous enhancement of the mass with delayed enhancement peripherally, due to fibrosis and central focal hypointensity due to necrosis. (e) First-pass perfusion imaging delineates the periphery of the mass. (f) Graphic representation of the dynamic enhancement demonstrates contrast enhancement in the lesion core (green) when compared with the lung (blue).

Figure 3.

Immunohistochemistry shows tumor cells strongly positive for vimentin.