title: Rare presentation of four primary pediatric cardiac tumors.
abstract: 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.
authors.affiliation: Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US.
authors.forename: Lauren R
authors.initials: LR
authors.lastname: Schaffer
authors.affiliation: Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US.
authors.forename: Shelley A
authors.initials: SA
authors.lastname: Caltharp
authors.affiliation: Department of Radiology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.
authors.forename: Sarah S
authors.initials: SS
authors.lastname: Milla
authors.affiliation: Department of Pediatric Cardiac Surgery, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.
authors.forename: Brian F
authors.initials: BF
authors.lastname: Kogon
authors.affiliation: Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US.
authors.forename: Caitlin A
authors.initials: CA
authors.lastname: Cundiff
authors.affiliation: Department of Pediatric Cardiology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.
authors.forename: Aarti
authors.initials: A
authors.lastname: Dalal
authors.affiliation: Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US.
authors.forename: Phillip C
authors.initials: PC
authors.lastname: Quigley
authors.affiliation: Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US. Electronic address: bshehat@emory.edu.
authors.forename: Bahig M
authors.initials: BM
authors.lastname: Shehata
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D002648:Child; D004452:Echocardiography; D017809:Fatal Outcome; D005354:Fibrosarcoma / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006338:Heart Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D015618:Histiocytosis, Sinus / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D015470:Leukemia, Myeloid, Acute / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D008297:Male; D009206:Myocardium / Q000737:chemistry / Q000473:pathology*; D013584:Sarcoma, Synovial / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Cardiac tumors; Congenital infantile fibrosarcoma of the heart; Myeloid sarcoma; Pediatric; Primary cardiac synovial sarcoma; Rosai Dorfman Disease