학술논문

Case report: Cardiac intimal sarcoma in a young child
Document Type
article
Source
Frontiers in Pediatrics, Vol 11 (2023)
Subject
intimal sarcoma
cardiac
child
MDM2
right ventricle (RV)
Pediatrics
RJ1-570
Language
English
ISSN
2296-2360
Abstract
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.