From the Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104 (M.M.P.); Sections of Thoracic Radiology (J.P.L.) and Pediatric Radiology (D.M.B.), American College of Radiology Institute of Radiologic Pathology, Silver Spring, Md; Department of Radiology, George Washington University, Washington, DC (J.P.L.); Department of Pathology, University of South Carolina School of Medicine, Greenville, SC (A.R.H.); Department of Pathology, MD Anderson Cancer Center, Houston, Tex (M.G.); and Department of Radiology (K.I.R.S., A.F., A.M.W., D.M.B.) and Division of Cardiology (D.V.), Children's Hospital of Philadelphia, Philadelphia, Penn.
Radiographics. 2023 Sep;43(9):e230010. doi: 10.1148/rg.230010.
Primary cardiac and pericardial neoplasms are rare in the pediatric population and can include both benign and malignant lesions. Rhabdomyomas, teratomas, fibromas, and hemangiomas are the most common benign tumors. The most common primary cardiac malignancies are soft-tissue sarcomas, including undifferentiated sarcomas, rhabdomyosarcomas, and fibrosarcomas. However, metastatic lesions are more common than primary cardiac neoplasms. Children with primary cardiac and pericardial tumors may present with nonspecific cardiovascular symptoms, and their clinical presentation may mimic that of more common nonneoplastic cardiac disease. The diagnosis of cardiac tumors has recently been facilitated using noninvasive cardiac imaging. Echocardiography is generally the first-line modality for evaluation. Cardiac MRI and CT are used for tissue characterization and evaluation of tumor size, extension, and physiologic effect. The varied imaging appearances of primary cardiac neoplasms can be explained by their underlying abnormality. Treatment of these lesions varies from conservative management, with spontaneous regression of some lesions such as rhabdomyomas, to surgical resection, particularly in patients with associated heart failure. With adequate imaging techniques and knowledge of the pathologic basis of the neoplasm, it is often possible to differentiate benign from malignant tumors, which can greatly affect adequate and timely treatment. RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.
原发性心脏和心包肿瘤在儿科人群中较为罕见，可包括良性和恶性病变。横纹肌瘤、畸胎瘤、纤维瘤和血管瘤是最常见的良性肿瘤。最常见的原发性心脏恶性肿瘤是软组织肉瘤，包括未分化肉瘤、横纹肌肉瘤和纤维肉瘤。然而，转移性病变比原发性心脏肿瘤更为常见。患有原发性心脏和心包肿瘤的儿童可能表现出非特异性心血管症状，其临床表现可能类似于更常见的非肿瘤性心脏疾病。最近，非侵入性心脏成像技术有助于心脏肿瘤的诊断。超声心动图通常是评估的首选方式。心脏 MRI 和 CT 用于组织特征分析以及评估肿瘤的大小、范围和生理影响。原发性心脏肿瘤的不同影像学表现可以用其潜在的异常来解释。这些病变的治疗方法各不相同，从保守治疗（一些病变如横纹肌瘤可自发消退）到手术切除，特别是在伴有心力衰竭的患者中。通过适当的成像技术和对肿瘤病理基础的了解，通常可以区分良性和恶性肿瘤，这对获得适当和及时的治疗非常重要。RSNA，2023 本文的在线学习中心提供测验题。
