Rare presentation of four primary pediatric cardiac tumors

Lauren R. Schaffer, Shelley A. Caltharp, Sarah S. Milla, Brian F. Kogon, Caitlin A. Cundiff, Aarti Dalal, Phillip C. Quigley, Bahig M. Shehata

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


Pediatric cardiac tumors are extremely rare and usually benign. We selected four unique cases of pediatric cardiac tumors from a 15-year period at our institution. The four chosen cases represent unique, rare primary tumors of the heart. Our selection includes a case of Rosai Dorfman disease without systemic involvement, which is, to our knowledge, the second case of isolated cardiac Rosai Dorfman disease in a child. We present a case of subtotal replacement of myocardium by granulocytic sarcoma with minimal bone marrow involvement, representing the first reported case in a child manifested as hypertrophic cardiomyopathy, as well as a case of a primary synovial sarcoma arising from the atrioventricular (AV) node, representing the fourth reported pediatric case of a cardiac synovial sarcoma, and it is the first to arise from the AV node. Finally, we present a primary congenital infantile fibrosarcoma of the heart, which is, to our knowledge, the first confirmed cardiac congenital infantile fibrosarcoma. These four cases represent the need for continued inclusion of rare cardiac conditions in a clinician's differential diagnosis. Furthermore, they present the need for more in-depth molecular and genomic analysis of pediatric cardiac tumors in order to identify their etiopathogenesis.

Original languageEnglish
Pages (from-to)72-77
Number of pages6
JournalCardiovascular Pathology
Issue number1
StatePublished - Jan 1 2016


  • Cardiac tumors
  • Congenital infantile fibrosarcoma of the heart
  • Myeloid sarcoma
  • Pediatric
  • Primary cardiac synovial sarcoma
  • Rosai Dorfman Disease


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