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Case 9: Pulmonary Artery Sarcoma Mimicking Massive Thromboembolic Disease

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Part of the book series: Clinical Cases in Cardiology ((CCC))

Abstract

Large-vessel pulmonary artery sarcomas are rare tumors of the pulmonary vasculature, accounting for only a fraction of primary lung cancers and with a very poor prognosis. They arise proximally from within the pulmonary artery and spread within the arterial lumen, causing obstruction of the vasculature that may result in right ventricular failure. Although they may have a similar presentation to chronic thromboembolic pulmonary disease and CTEPH, there are subtle indicators in history and imaging that can help with their differential diagnosis, with the confirmation usually resulting from tissue biopsy. This case presentation focuses on this CTEPH mimic.

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Further Reading

  • Gan H-L, Zhang J-Q, Huang X-Y, Yu W. The wall eclipsing sign on pulmonary artery computed tomography is pathognomonic for pulmonary artery sarcoma. PLoS One. 2013;8(12):e83200.

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  • Grazioli V, Vistarini N, Morsolini M, et al. Surgical treatment of primary pulmonary artery sarcoma. J Thorac Cardiovasc Surg. 2014;148:113–8.

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  • Mussot S, Ghigna MR, Mercier O, et al. Retrospective institutional study of 31 patients, treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg. 2013;43:787–93.

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  • Papamatheakis DG, Kerr KM. Pulmonary vascular tumors. In: Peacock AJ, Naeije R, Rubin LJ, editors. Pulmonary circulation. Diseases and their treatment. Boca Raton, FL: CRC Press (Taylor & Francis group; 2016. p. 736–42.

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  • von Falck C, Meyer B, Fegbeutel C, et al. Imaging features of primary sarcomas of the great vessels in CT, MRI and PET/CT: a single center experience. BMC Med Imaging. 2013;13:25.

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Correspondence to Demosthenes G. Papamatheakis .

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Papamatheakis, D.G., Gopalan, D., Auger, W.R. (2020). Case 9: Pulmonary Artery Sarcoma Mimicking Massive Thromboembolic Disease. In: Auger, W., Gopalan, D. (eds) Clinical Cases in Chronic Thromboembolic Pulmonary Hypertension. Clinical Cases in Cardiology. Springer, Cham. https://doi.org/10.1007/978-3-030-17366-1_9

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  • DOI: https://doi.org/10.1007/978-3-030-17366-1_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-17365-4

  • Online ISBN: 978-3-030-17366-1

  • eBook Packages: MedicineMedicine (R0)

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