Case report: Cardiac intimal sarcoma in a young child

Sanne Verbeek; Raf Sciot; Maria Debiec-Rychter; Veerle Labarque; Bart Meyns; Bjorn Cools

doi:10.3389/fped.2023.1238847

Case report: Cardiac intimal sarcoma in a young child

Front Pediatr. 2023 Sep 25:11:1238847. doi: 10.3389/fped.2023.1238847. eCollection 2023.

Authors

Sanne Verbeek¹, Raf Sciot¹, Maria Debiec-Rychter², Veerle Labarque³, Bart Meyns⁴, Bjorn Cools⁵

Affiliations

¹ Department of Pathology, University Hospitals Leuven, Leuven, Belgium.
² Center for Human Genetics, Catholic University Leuven, Leuven, Belgium.
³ Department of Pediatric Hematology and Oncology, University Hospitals Leuven, Leuven, Belgium.
⁴ Department of Cardiac Surgery, University Hospitals Leuven, Leuven, Belgium.
⁵ Department of Pediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium.

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.

Keywords: MDM2; cardiac; child; intimal sarcoma; right ventricle (RV).

Publication types

Case Reports