AUTHOR=Verbeek Sanne , Sciot Raf , Debiec-Rychter Maria , Labarque Veerle , Meyns Bart , Cools Bjorn 

TITLE=Case report: Cardiac intimal sarcoma in a young child

JOURNAL=Frontiers in Pediatrics

VOLUME=11

YEAR=2023

URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2023.1238847

DOI=10.3389/fped.2023.1238847

ISSN=2296-2360

ABSTRACT=<p>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 <italic>MDM2</italic> gene and a homozygous loss of <italic>CDKN2A</italic> on 9p21. A review of the literature regarding primary cardiac malignancies and intimal sarcoma in children is provided.</p>