PMC5457927_01
Radiological examinations. (A) Contrast-enhanced computed tomography (CT) shows a soft-tissue density mass in the right distal main pulmonary artery. (B) 18F-fluorodeoxyglucose positron emission tomography with integrated CT shows the mass with a high uptake of 18F-fluorodeoxyglucose in the right distal main pulmonary artery. (C) A multiplanar reconstruction image (oblique plane) shows an intravascular mass within the right distal main pulmonary artery that extends to the descending interlobar artery and the segmental branches.
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Contrast-enhanced chest CT (Fig. 1A) and 18F-fluorodeoxyglucose PET/CT (Fig. 1B) revealed a 2.7-cm, soft-tissue density mass with high uptake of 18F-fluorodeoxyglucose in the right distal main pulmonary artery. A multiplanar reconstruction image (oblique plane, Fig. 1C) showed the mass within the right distal main pulmonary artery that extends to the descending interlobar artery and the segmental branches.
Angiographic examinations. (A) Three-dimensional computed tomographic angiography (posterior oblique view) shows complete occlusion of the right distal main pulmonary artery (arrow). (B) pulmonary angiography shows a filling defect occupying the entire lumen of the right distal main pulmonary artery (arrowhead). (C) An endovascular catheter biopsy using forceps is performed following pulmonary angiography.
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Three-dimensional CT angiography (posterior oblique view, Fig. 2A) and pulmonary angiography (Fig. 2B) showed a large filling defect in the right distal main pulmonary artery adjacent to the right upper lobe artery, and the descending interlobar artery was completely occluded by the lesion. An endovascular catheter biopsy using forceps for myocardial biopsy was then performed (Fig. 2C).
Histological examinations. (A) The tumor is composed of fascicular and poorly-arranged proliferation of atypical spindle cells. The tumor cells are anaplastic, with atypical nuclei and some multinucleated cells (Hematoxylin and Eosin staining, original magnification 200x). (B) The tumor cells are immunohistochemically positive for platelet-derived growth factor receptor alpha (original magnification 200x).
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The biopsy specimens showed fascicular proliferation of spindle cells with pleomorphic nuclei in a background of fibrin and necrosis (Fig. 3A). Further immunohistochemical examination using the PDGFRalpha antibody (catalogue number SC338, Santa Cruz) showed that more than 50% of the tumor cells were positive for PDGFRalpha (Fig. 3B).