Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease

Abstract

Two cases of primary pulmonary artery sarcoma resembling chronic thromboembolic disease features are presented. Tumour identification was achieved after pulmonarv thromboendarterectomy, which was indicated by documentation of a prothrombotic state in both patients. A doubtful history of pulmonary emboli or deep venous thrombosis should alert medical personnel to the possible presence of a primary pulmonary artery sarcoma. Advanced imaging methods such as gadolinium-enhanced magnetic resonance imaging could be useful in considering pulmonary thromboendarterectomy. If a tumour is detected, its surgical resection should be considered with caution, taking into account the poor survival results. Invasion of the adventitia or the right ventricle, as documented in the present cases, is unusual. As far as the present authors know, this is the first report of this kind of tumour and its coexistence with an activated protein C resistance state and type II heparin-induced thrombocytopenia.

Diagnostic usefulness of 18F-FDG PET/CT in the differentiation of pulmonary artery sarcoma and pulmonary embolism

“…PAS is a rare malignancy arising from mesenchymal cells of the intima of the pulmonary artery that is frequently misdiagnosed as a pulmonary thromboembolism because the clinical symptoms and radiological findings are similar [3][4][5]. According to a previous study focusing on the chest CT findings, PAS can be distinguished from PE by the presence of a low-attenuation filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery, expansion of the involved arteries, extraluminal tumor extension, laterality of the lesion and metastasis [6,7].…”

“…Both diseases have similar clinical findings, causing severe dyspnea and chronic hypoxemia [2]. Physicians frequently misdiagnose PAS as PE in their physical findings and on conventional CT images [3][4][5]. A few cases have suggested that contrast-enhanced CT can distinguish PAS from PE by visualizing extraluminal tumor extension, expansion of the involved arteries, or a low attenuation-filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery [6][7][8].…”

Pulmonary Artery Sarcoma: A Histologic and Follow-up Study With Emphasis on a Subset of Low-grade Myofibroblastic Sarcomas With a Good Long-term Follow-up

“…2,6,14,21,23 Many pulmonary artery sarcomas are initially diagnosed as recurrent pulmonary embolism with thromboembolic pulmonary hypertension, as in this study. 1,12,15,[18][19][20]25,[27][28][29] It has been shown that imaging features, especially inhomogeneous high or low attenuation, soft-tissue density in pulmonary arteries, vascular distension, and enhancement after administration of gadopentetate dimeglumine are reliable in distinguishing recurrent pulmonary embolism from primary sarcoma of the artery. 11,16 The histologic classification of pulmonary artery sarcomas is variable with some authors emphasizing the presumed intimal origin, 6 whereas others use classifications of soft tissue neoplasms.…”

The Role of Radiation Diagnostic Techniques in the Diagnosis of Chronic Thromboembolic Pulmonary Hypertension

“…Выполнение КТА позволяет также исклю-чить другие причины обструкции ЛА, такие как фиброзирующий медиастинит, новообра-зование средостения и саркому [22].…”