Presentation
Productive cough and progressively worsening shortness of breath over the past 6 months. She has a 30-pack-year smoking history.
Patient Data
There are numerous extensive filling defects, the largest of which is a branching filling defect extending from the pulmonary arterial bifurcation nearly completely occluding the left pulmonary artery. There is an extension into numerous lobar and segmental branches in the upper and lower lobes, particularly the upper lobe. There are similar but smaller filling defects in the distal right pulmonary artery extending into the right upper and lower lobar branches and the segmental and subsegmental branches, respectively. Many of these filling defects expand the vessel, particularly in the left upper lobe. There are numerous smaller peripheral filling defects bilaterally.
There is mildly prominent soft tissue along pulmonary arterial structures at both hila, probably a combination of soft tissue within the vessels as well as mildly prominent lymph tissue, but discrete nodes are difficult to visualize. There is an enlarged lymph node at the AP window.
The main pulmonary artery is normal in caliber.
There is a large soft tissue mass seen within the left pulmonary artery. On the fused PET/CT, there is increased radiotracer activity corresponding to this soft tissue mass and, to a lesser extent, the right pulmonary artery. On the left, there is an extension of radiotracer activity into the lobar and segmental branches.
There is an avid AP window lymph node.
There is no axillary, abdominal, or pelvic lymphadenopathy
Case Discussion
This is a case of sarcoma within the left pulmonary artery. A CT-guided biopsy of the mass was performed. Histopathology revealed markedly atypical cellular elements that were epithelioid to polygonal in shape, generally isolated without aggregation or nesting, and with a variable amount of cytoplasm. Immunohistochemistry stains demonstrated positivity for vimentin only suggesting a diagnosis of sarcoma.
The patient has received several cycles of chemotherapy. Follow-up imaging has shown an interval decrease in the size of the mass, as well as decreased extension into the pulmonary artery branches.
Co-author:
Camille Dumas, DO