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A Rare Case of an Intracardiac Myoepithelial Carcinoma in an Infant

Patel, Priyal, DO*; Roberts, Ryan, MD, PhD*; Cole, Jason, MD; Stalling, Melissa, DO; Nicol, Kathleen, MD; Hor, Kan, MD§; Bowman, Jessica, MD; Setty, Bhuvana, MD*

Journal of Pediatric Hematology/Oncology: May 2019 - Volume 41 - Issue 4 - p e206–e209
doi: 10.1097/MPH.0000000000001343
Online Articles: Original Articles

Myoepithelial carcinomas (MC) represent aggressive tumors that occur in a myriad of ages and anatomic locations. The rarity and histologic similarity with other tumors make them difficult to diagnosis. We report an extremely rare case of a right ventricular outflow tract mass identified to be an intracardiac MC in a 4-month-old male infant. Pathology revealed an EWS-KLF15 translocation. Treatment included gross total resection and intensive chemotherapy. Recurrent cardiac mass with brain metastasis was seen 16 months after primary diagnosis. We describe the rarity of intracardiac MC in pediatric patients and the challenges encountered in the multimodal management of this patient.

*Department of Pediatrics, Division of Pediatric Hematology/Oncology/BMT

Division of Pathology

Division of Cardiology

§Division of Radiology, Nationwide Children’s Hospital, Columbus, OH

The authors declare no conflict of interest.

Reprints: Bhuvana Setty, MD, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH 43205 (e-mail:

Received February 28, 2018

Accepted September 16, 2018

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.