Abstract
BackgroundThe objective of this study was to evaluate the imaging characteristics of pulmonary artery sarcoma (PAS) on pulmonary artery computed tomography angiography (PACTA) that can be used to differentiate between PAS and pulmonary thromboembolic diseases, including chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (APE).MethodsThe clinical data and imaging characteristics of 12 patients with PAS, 156 patients with CTEPH, and 426 patients with APE who were treated at Beijing Anzhen Hospital from January 2007 to August 2013 were retrospectively analyzed. All patients underwent PACTA before treatment, and the diagnoses of PAS and CTEPH were all confirmed by surgical biopsy.ResultsAll 12 PAS patients were initially misdiagnosed and received inappropriate thrombolytic and/or anticoagulant therapy before they were referred for surgical intervention. The mean time from PACTA to surgical intervention was 5.5±3.7 months (range 2–11 months). On PACTA, the PAS lesion always eclipsed the wall of the pulmonary artery before infiltrating outside the pulmonary artery, which was termed the wall eclipsing sign. This sign was observed in all PAS patients but was not observed in any CTEPH or APE patients.ConclusionsPAS is a rare neoplasm with a poor prognosis, and is easily misdiagnosed as thromboembolic disease. The wall eclipsing sign on PACTA is pathognomonic for PAS, and patients with this sign should be investigated to confirm the diagnosis and should undergo surgical intervention as soon as possible, rather than receiving thrombolytic or anticoagulant therapy.
Highlights
Pulmonary artery sarcoma (PAS) is an extremely rare neoplasm that is usually indistinguishable from acute or chronic thromboembolic disease of the pulmonary arteries on clinical and radiological findings
In patients with pulmonary artery sarcoma (PAS), the mean lactate dehydrogenase (LDH) level, high-sensitivity C-reactive protein level, B-type natriuretic peptide (BNP) level, and erythrocyte sedimentation rate (ESR) were all elevated, but the mean D-Dimer level was in the normal range
In patients with chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (APE), the mean LDH level, hs-CRP level, and ESR were in the normal range, but the mean BNP level was elevated
Summary
Pulmonary artery sarcoma (PAS) is an extremely rare neoplasm that is usually indistinguishable from acute or chronic thromboembolic disease of the pulmonary arteries on clinical and radiological findings. The clinical manifestations of PAS are non-specific and very similar to those of thromboembolic disease, resulting in frequent delays in making the correct diagnosis and initiating proper treatment. The incidence of PAS is very low, this disease should be included in the differential diagnosis of pulmonary thromboembolism, especially in patients who do not respond to thrombolytic/anticoagulant therapy or who present with no identifiable source for thromboembolic events [3]. The objective of this study was to evaluate the imaging characteristics of pulmonary artery sarcoma (PAS) on pulmonary artery computed tomography angiography (PACTA) that can be used to differentiate between PAS and pulmonary thromboembolic diseases, including chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (APE)
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