PMC5496329_01
Contrast enhanced CT imaging of the thorax (a, b axial; c, d oblique coronal; e sagittal reformatted images) shows a hypodense filling defect (asterisk) in the main and bilateral pulmonary arteries which does not exhibit post contrast enhancement (MPA main pulmonary artery, RPA right pulmonary artery, LPA left pulmonary artery). No pulmonary parenchymal abnormality was noted
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Fig1
A contrast enhanced CT was performed which revealed a hypodense filling defect in the main and bilateral pulmonary arteries which did not exhibit any significant post contrast enhancement (Fig. 1).
a Sarcomatous tumor with intratumoral haemorrhage and fibrinoid necrosis [Haematoxylin and Eosin (H and E) stain x200]. b Higher magnification showing tumor cells with focal epithelioid morphology, exhibiting moderate to marked nuclear pleomorphism [H and E x400]. c By Immunohistochemistry, tumor cells showing diffuse intranuclear Fli1 positivity (diaminobenzidine immunostain x400)
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Fig2
On Immunohistochemistry, tumor cells were diffusely positive for Fli1 (Fig. 2c), which is a surrogate vascular marker; focally weakly positive for CD31, while were negative for CD34.
Post pulmonary endarterectomy cine-cardiac MRI (axial a, b, c, d T1W; b STIR, T2 and Post-contrast respectively) shows residual disease (asterisk) in the left pulmonary trunk with exophytic extravascular component. Focal patchy consolidation like opacities (arrow) were also seen in the lung parenchyma which were in favour of chronic thromboembolic phenomenon related infarcts presenting as consolidation (confirmed on post-contrast CT thorax: e mediastinal window and f lung window)
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Fig3
Cine-cardiac MRI was performed which showed non-enhancing altered signal intensity lobulated mass in the left pulmonary artery with both exophytic and endophytic intravascular component (Fig. 3a-d). CT was performed to rule out pulmonary metastatic disease however CT thorax revealed ill-defined focal patchy pneumonic areas in bilateral lung parenchyma that were in favour of pulmonary changes as a sequel to chronic thromboembolic phenomenon (Fig. 3e, f).
Follow-up imaging after 12 cycles of radiotherapy shows a large lobulated peripherally enhancing exophytic mediastinal mass (asterisk) arising from the left main pulmonary artery and invading its segmental arteries with a well-defined soft tissue nodule in left lower (arrow). CT findings were suggestive of disease progression with pulmonary parenchymal metastasis
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Fig4
Follow-up imaging after 12 cycles of radiotherapy revealed significant progression of the primary disease with new development of pulmonary parenchymal metastasis (Fig. 4).