Case report: Cardiac intimal sarcoma in a young child

Undifferentiated mesenchymal tumors from the intimal layer (intimal sarcomas) are rare within the ventricles and exceptional in children. A rare case of an intimal sarcoma located in the right ventricle in a young child is presented with need for urgent surgical resection due to mechanical flow obst...

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Main Authors: Sanne Verbeek, Raf Sciot, Maria Debiec-Rychter, Veerle Labarque, Bart Meyns, Bjorn Cools
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-09-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fped.2023.1238847/full
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author Sanne Verbeek
Raf Sciot
Maria Debiec-Rychter
Veerle Labarque
Bart Meyns
Bjorn Cools
author_facet Sanne Verbeek
Raf Sciot
Maria Debiec-Rychter
Veerle Labarque
Bart Meyns
Bjorn Cools
author_sort Sanne Verbeek
collection DOAJ
description Undifferentiated mesenchymal tumors from the intimal layer (intimal sarcomas) are rare within the ventricles and exceptional in children. A rare case of an intimal sarcoma located in the right ventricle in a young child is presented with need for urgent surgical resection due to mechanical flow obstruction. Tumor cells showed amplification of MDM2 gene and a homozygous loss of CDKN2A on 9p21. A review of the literature regarding primary cardiac malignancies and intimal sarcoma in children is provided.
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spelling doaj.art-7e9e32199c234de2a6d0ac50c9d29d362023-09-26T04:54:57ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-09-011110.3389/fped.2023.12388471238847Case report: Cardiac intimal sarcoma in a young childSanne Verbeek0Raf Sciot1Maria Debiec-Rychter2Veerle Labarque3Bart Meyns4Bjorn Cools5Department of Pathology, University Hospitals Leuven, Leuven, BelgiumDepartment of Pathology, University Hospitals Leuven, Leuven, BelgiumCenter for Human Genetics, Catholic University Leuven, Leuven, BelgiumDepartment of Pediatric Hematology and Oncology, University Hospitals Leuven, Leuven, BelgiumDepartment of Cardiac Surgery, University Hospitals Leuven, Leuven, BelgiumDepartment of Pediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, BelgiumUndifferentiated mesenchymal tumors from the intimal layer (intimal sarcomas) are rare within the ventricles and exceptional in children. A rare case of an intimal sarcoma located in the right ventricle in a young child is presented with need for urgent surgical resection due to mechanical flow obstruction. Tumor cells showed amplification of MDM2 gene and a homozygous loss of CDKN2A on 9p21. A review of the literature regarding primary cardiac malignancies and intimal sarcoma in children is provided.https://www.frontiersin.org/articles/10.3389/fped.2023.1238847/fullintimal sarcomacardiacchildMDM2right ventricle (RV)
spellingShingle Sanne Verbeek
Raf Sciot
Maria Debiec-Rychter
Veerle Labarque
Bart Meyns
Bjorn Cools
Case report: Cardiac intimal sarcoma in a young child
Frontiers in Pediatrics
intimal sarcoma
cardiac
child
MDM2
right ventricle (RV)
title Case report: Cardiac intimal sarcoma in a young child
title_full Case report: Cardiac intimal sarcoma in a young child
title_fullStr Case report: Cardiac intimal sarcoma in a young child
title_full_unstemmed Case report: Cardiac intimal sarcoma in a young child
title_short Case report: Cardiac intimal sarcoma in a young child
title_sort case report cardiac intimal sarcoma in a young child
topic intimal sarcoma
cardiac
child
MDM2
right ventricle (RV)
url https://www.frontiersin.org/articles/10.3389/fped.2023.1238847/full
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