Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles

Leiomyosarcoma of the pulmonary vein is rare and has poor prognosis. Its clinical features are nonspecific and mimic benign conditions. Early diagnosis is challenging. Most cases have been diagnosed only at autopsy or on postoperative histology specimens. Treatment is essentially palliative complete...

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Main Authors: Philemon Gukop, Guido Frassetto, Georgios Karapanagiotidis, Venkatachalam Chandrasekaran
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Cardiology
Online Access:http://dx.doi.org/10.1155/2012/396319
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author Philemon Gukop
Guido Frassetto
Georgios Karapanagiotidis
Venkatachalam Chandrasekaran
author_facet Philemon Gukop
Guido Frassetto
Georgios Karapanagiotidis
Venkatachalam Chandrasekaran
author_sort Philemon Gukop
collection DOAJ
description Leiomyosarcoma of the pulmonary vein is rare and has poor prognosis. Its clinical features are nonspecific and mimic benign conditions. Early diagnosis is challenging. Most cases have been diagnosed only at autopsy or on postoperative histology specimens. Treatment is essentially palliative complete surgical excision. We outline the principles of management with the case of a 39-year-old man with leiomyosarcoma of the left pulmonary veins extending into the left atrium. Extensive investigation to achieve early diagnosis and determine extent of disease is essential. Frozen section guided adequate excision of all cardiac tumours and resection of involved lung tissue achieve local disease control. Adjuvant chemoradiotherapy has been shown to enhance survival.
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institution Kabale University
issn 2090-6404
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publishDate 2012-01-01
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series Case Reports in Cardiology
spelling doaj-art-1ae6dcea9cca4fd8a928b51569158bae2025-02-03T06:14:16ZengWileyCase Reports in Cardiology2090-64042090-64122012-01-01201210.1155/2012/396319396319Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management PrinciplesPhilemon Gukop0Guido Frassetto1Georgios Karapanagiotidis2Venkatachalam Chandrasekaran3Department of Cardiothoracic Surgery, St George’s Hospital, London SW17 0QT, UKDepartment of Cardiothoracic Surgery, St George’s Hospital, London SW17 0QT, UKDepartment of Cardiothoracic Surgery, St George’s Hospital, London SW17 0QT, UKDepartment of Cardiothoracic Surgery, St George’s Hospital, London SW17 0QT, UKLeiomyosarcoma of the pulmonary vein is rare and has poor prognosis. Its clinical features are nonspecific and mimic benign conditions. Early diagnosis is challenging. Most cases have been diagnosed only at autopsy or on postoperative histology specimens. Treatment is essentially palliative complete surgical excision. We outline the principles of management with the case of a 39-year-old man with leiomyosarcoma of the left pulmonary veins extending into the left atrium. Extensive investigation to achieve early diagnosis and determine extent of disease is essential. Frozen section guided adequate excision of all cardiac tumours and resection of involved lung tissue achieve local disease control. Adjuvant chemoradiotherapy has been shown to enhance survival.http://dx.doi.org/10.1155/2012/396319
spellingShingle Philemon Gukop
Guido Frassetto
Georgios Karapanagiotidis
Venkatachalam Chandrasekaran
Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles
Case Reports in Cardiology
title Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles
title_full Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles
title_fullStr Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles
title_full_unstemmed Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles
title_short Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles
title_sort leiomyosarcoma of pulmonary vein presenting as left atrial mass an outline of management principles
url http://dx.doi.org/10.1155/2012/396319
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