Clinical and radiological features of pulmonary artery sarcoma: A report of nine cases

Abstract

The clinical features of PAS that may help to differentiate it from PTD are its insidious onset, constitutional symptoms, increased serum inflammatory marker levels, lack of predisposing factors and unresponsiveness to thrombolytic and/or anticoagulation therapy. Typical CTPA features are crucial for suspicion of PAS. Early biopsy or surgery should be performed to confirm the diagnosis of PAS and improve its prognosis.

Pulmonary artery intimal sarcoma: a case report and literature review

“…Constitutional symptoms such as fever, fatigue, and weight loss are the features of malignancy. These could help in differentiating pulmonary artery sarcoma from other aetiologies of right ventricular failure. However, physicians seldom establish a diagnosis of pulmonary intimal artery sarcoma based on symptoms alone.…”

“…Other investigational modalities are usually required. From published studies, the modalities that have been used are echocardiography, CT, magnetic resonance imaging (MRI), and positron emission tomography (PET) .…”

“…In a cohort of nine patients, transthoracic echocardiography provided information about the functioning of the pulmonary valve and right ventricle. However, none of the cases were diagnosed as pulmonary artery sarcoma solely on echocardiography . Other radiological modalities are therefore inevitably conducted for confirmation of the diagnosis.…”

“…The radiological features of pulmonary artery sarcoma are filling defect in the pulmonary artery lumen, extraluminal tumour invasion, and a nodular appearance. However, despite specific features, published studies have shown that more than half of the patients with pulmonary artery sarcoma are initially misdiagnosed as pulmonary thromboembolism . Several studies have been conducted in an attempt to distinguish between the two entities.…”

“…Thus, the CT features are characteristic with expansive growth and a bulging appearance against the direction of blood flow, a lobulated structure at the proximal part of the tumours, a grape‐like appearance at the distal end of the tumours, and heterogeneous enhancement . Pulmonary artery sarcoma can extend until the bifurcation of the pulmonary arteries, the main pulmonary artery, pulmonary valve, and right ventricular outflow tract . In contrast, the proximal end of a chronic pulmonary thromboembolus is a straight and cup‐like structure, caused by blood flow against the surface of blood clots .…”