Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report

Pulmonary artery intimal sarcoma (PAIS) is an extremely rare malignant tumour. It is often misdiagnosed as chronic pulmonary thromboembolism. We describe a complex case in a 70-year-old man with PAIS extending into his right ventricle undergoing salvage cytoreductive surgical resection utilizing biv...

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Autores principales: Williams, Michael L, Tan, Charis, Misfeld, Martin, Yan, Tristan D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8007161/
https://www.ncbi.nlm.nih.gov/pubmed/33815748
http://dx.doi.org/10.1093/jscr/rjab051
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author Williams, Michael L
Tan, Charis
Misfeld, Martin
Yan, Tristan D
author_facet Williams, Michael L
Tan, Charis
Misfeld, Martin
Yan, Tristan D
author_sort Williams, Michael L
collection PubMed
description Pulmonary artery intimal sarcoma (PAIS) is an extremely rare malignant tumour. It is often misdiagnosed as chronic pulmonary thromboembolism. We describe a complex case in a 70-year-old man with PAIS extending into his right ventricle undergoing salvage cytoreductive surgical resection utilizing bivalirudin for cardiopulmonary bypass anticoagulation due to heparin-induced thrombocytopenia and thrombosis syndrome. The prognosis for PAIS is extremely poor, with a median survival of 1.5 months without surgical resection. Cytoreductive surgical debulking can improve the median survival time to 17 months. The main aim of palliative surgical resection is to improve ventilation–perfusion mismatch and prevent haemodynamic collapse.
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spelling pubmed-80071612021-04-02 Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report Williams, Michael L Tan, Charis Misfeld, Martin Yan, Tristan D J Surg Case Rep Case Report Pulmonary artery intimal sarcoma (PAIS) is an extremely rare malignant tumour. It is often misdiagnosed as chronic pulmonary thromboembolism. We describe a complex case in a 70-year-old man with PAIS extending into his right ventricle undergoing salvage cytoreductive surgical resection utilizing bivalirudin for cardiopulmonary bypass anticoagulation due to heparin-induced thrombocytopenia and thrombosis syndrome. The prognosis for PAIS is extremely poor, with a median survival of 1.5 months without surgical resection. Cytoreductive surgical debulking can improve the median survival time to 17 months. The main aim of palliative surgical resection is to improve ventilation–perfusion mismatch and prevent haemodynamic collapse. Oxford University Press 2021-03-29 /pmc/articles/PMC8007161/ /pubmed/33815748 http://dx.doi.org/10.1093/jscr/rjab051 Text en Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2021. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Williams, Michael L
Tan, Charis
Misfeld, Martin
Yan, Tristan D
Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
title Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
title_full Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
title_fullStr Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
title_full_unstemmed Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
title_short Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
title_sort cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8007161/
https://www.ncbi.nlm.nih.gov/pubmed/33815748
http://dx.doi.org/10.1093/jscr/rjab051
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