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Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract

BACKGROUND: Primary undifferentiated spindle cell sarcoma in the right ventricle is an extremely rare tumor. Radical surgical excision is the optimal treatment for long-term survival due to poor response to chemotherapy or radiotherapy at an advanced stage. CASE PRESENTATION: A 42-year-old man with...

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Autores principales: Kwon, Jintae, Kwon, Kyewon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7199296/
https://www.ncbi.nlm.nih.gov/pubmed/32366268
http://dx.doi.org/10.1186/s13019-020-01129-8
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author Kwon, Jintae
Kwon, Kyewon
author_facet Kwon, Jintae
Kwon, Kyewon
author_sort Kwon, Jintae
collection PubMed
description BACKGROUND: Primary undifferentiated spindle cell sarcoma in the right ventricle is an extremely rare tumor. Radical surgical excision is the optimal treatment for long-term survival due to poor response to chemotherapy or radiotherapy at an advanced stage. CASE PRESENTATION: A 42-year-old man with no previous medical history presented with mild dyspnea on exertion and abdominal distension that lasted a week. Computed tomography (CT) revealed a huge homogeneous mass completely obstructing the right ventricle and extending into the pulmonary trunk. However, he suddenly collapsed the next day while on his way to an echocardiography. An extracorporeal membrane oxygenation (ECMO) device was inserted percutaneously and ECMO support was urgently initiated. Based on consideration of right ventricular outflow tract (RVOT) obstruction in the initial CT scan, we decided to remove the mass from the right ventricle immediately. The main mass was resected to relieve the RVOT obstruction, and after the operation, the ECMO was removed from the operation room. However, the patient failed to regain consciousness and electroencephalography (EEG) and subsequent magnetic resonance imaging (MRI) indicated severe hypoxic brain damage. We assume CPR was unsuccessful because the mass completely blocked the RVOT. Pathology revealed the mass was an undifferentiated spindle cell sarcoma. CONCLUSIONS: We present the case of a 42-year-old male with cardiac arrest due to right ventricular outflow tract obstruction by a tumor of the right ventricle. Surgical resection was performed and in histopathology it was proved to be an undifferentiated spindle cell sarcoma.
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spelling pubmed-71992962020-05-08 Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract Kwon, Jintae Kwon, Kyewon J Cardiothorac Surg Case Report BACKGROUND: Primary undifferentiated spindle cell sarcoma in the right ventricle is an extremely rare tumor. Radical surgical excision is the optimal treatment for long-term survival due to poor response to chemotherapy or radiotherapy at an advanced stage. CASE PRESENTATION: A 42-year-old man with no previous medical history presented with mild dyspnea on exertion and abdominal distension that lasted a week. Computed tomography (CT) revealed a huge homogeneous mass completely obstructing the right ventricle and extending into the pulmonary trunk. However, he suddenly collapsed the next day while on his way to an echocardiography. An extracorporeal membrane oxygenation (ECMO) device was inserted percutaneously and ECMO support was urgently initiated. Based on consideration of right ventricular outflow tract (RVOT) obstruction in the initial CT scan, we decided to remove the mass from the right ventricle immediately. The main mass was resected to relieve the RVOT obstruction, and after the operation, the ECMO was removed from the operation room. However, the patient failed to regain consciousness and electroencephalography (EEG) and subsequent magnetic resonance imaging (MRI) indicated severe hypoxic brain damage. We assume CPR was unsuccessful because the mass completely blocked the RVOT. Pathology revealed the mass was an undifferentiated spindle cell sarcoma. CONCLUSIONS: We present the case of a 42-year-old male with cardiac arrest due to right ventricular outflow tract obstruction by a tumor of the right ventricle. Surgical resection was performed and in histopathology it was proved to be an undifferentiated spindle cell sarcoma. BioMed Central 2020-05-04 /pmc/articles/PMC7199296/ /pubmed/32366268 http://dx.doi.org/10.1186/s13019-020-01129-8 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Kwon, Jintae
Kwon, Kyewon
Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
title Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
title_full Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
title_fullStr Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
title_full_unstemmed Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
title_short Primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
title_sort primary undifferentiated spindle cell sarcoma obstructing the right ventricular outflow tract
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7199296/
https://www.ncbi.nlm.nih.gov/pubmed/32366268
http://dx.doi.org/10.1186/s13019-020-01129-8
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