Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pulmonary artery angiosarcoma
- Radiologic Findings
- Chest radiograph shows cardiomegaly with left hilar bulging shadow. Contrast-enhanced CT images show intraluminal soft tissue filling defects with peripheral enhancement and extraluminal tumor extension in pulmonary trunk and both main pulmonary arteries, and extension to right ventricular outflow track.
- Brief Review
- Pulmonary artery sarcoma is a rare malignancy arising from the mesenchymal cells of the intima of the pulmonary artery. It is usually presenting heterogenous histology that most likely reflect a pluripotential or mesenchymal cell origin. Some examples that have been reviewed in the literature represent undifferentiated sarcomas that may display a myofibroblastic or leiomyosarcomatous component; nevertheless, osteosarcomatous, chondrosarcomatous, rhabdomyosarcomatous, liposarcomatous and angiosarcomatous elements have also been described. It occurs in middle age, with no specific clinical features, which may mimic other diseases such as lung cancer, mediastinal masses, and especially acute or chronic pulmonary embolism. Symptoms include chest pain, cough, dyspnea, hemoptysis, pulmonary hypertension and right heart failure. CT findings favoring the diagnosis of pulmonary artery sarcoma include low-attenuation filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery, expansion of the involved arteries, and extraluminal tumor extension. MRI is highly specific in recognizing pulmonary artery sarcoma since the tumor enhances post-gadolinium contrast more than bland thrombus.
- References
- Am J Roentgenol. 2007;188 (6): 1691-3
- Keywords
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Vascular, Malignant tumor,