Schaffer Lauren R, Caltharp Shelley A, Milla Sarah S, Kogon Brian F, Cundiff Caitlin A, Dalal Aarti, Quigley Phillip C, Shehata Bahig M
Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US.
Department of Radiology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.
Cardiovasc Pathol. 2016 Jan-Feb;25(1):72-7. doi: 10.1016/j.carpath.2015.08.011. Epub 2015 Aug 31.
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.
小儿心脏肿瘤极为罕见,通常为良性。我们从本机构15年期间挑选出4例独特的小儿心脏肿瘤病例。所选的这4例病例代表了独特、罕见的原发性心脏肿瘤。我们的病例包括1例无全身累及的Rosai-Dorfman病,据我们所知,这是儿童孤立性心脏Rosai-Dorfman病的第二例。我们报告1例粒细胞肉瘤对心肌进行次全替代且骨髓累及极少的病例,这是首例表现为肥厚型心肌病的儿童病例,还有1例起源于房室结的原发性滑膜肉瘤病例,这是第四例报告的小儿心脏滑膜肉瘤病例,且是首例起源于房室结的病例。最后,我们报告1例原发性先天性婴儿心脏纤维肉瘤,据我们所知,这是首例确诊的先天性婴儿心脏纤维肉瘤。这4例病例表明临床医生在鉴别诊断中仍需纳入罕见的心脏疾病。此外,还需要对小儿心脏肿瘤进行更深入的分子和基因组分析,以确定其发病机制。