Padalino Massimo A, Reffo Elena, Cerutti Alessia, Favero Valentina, Biffanti Roberta, Vida Vladimiro, Stellin Giovanni, Milanesi Ornella
1 Department of Cardiac, Pediatric and Congenital Cardiac Surgery Unit, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy.
2 Department of Pediatrics, Division of Pediatric Cardiology, University of Padova Medical School, Padova, Italy.
Cardiol Young. 2014 Apr;24(2):268-74. doi: 10.1017/S104795111300022X. Epub 2013 Mar 5.
Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.
婴幼儿原发性心脏肿瘤极为罕见，根据超声心动图研究估计发病率为0.2%。由于其罕见性，相关文献非常少，大多数知识基于病例报告集。因此，我们回顾性分析了27年来我们在儿童心脏肿瘤整体管理方面的临床经验，不仅是为了增进我们的了解，也是为了向其他人提供有关这种罕见疾病的发病率、临床表现、管理和长期预后的信息。1982年4月至2009年4月期间，我们机构有52名儿童被诊断患有心脏肿瘤。我们研究了病历和随访超声心动图评估。35%的患者在产前被诊断。最常见的肿瘤类型是横纹肌瘤（61.5%）、纤维瘤（15.4%）和黏液瘤（5.8%）。没有原发性恶性肿瘤病例。所有诊断均通过二维超声心动图完成，12名患者进行了病理分析。共有41名患者（79%）接受了药物治疗，而11名患者（21%）接受了手术治疗。平均随访7.2±5.4年，两名患者死于与心脏移植相关的并发症；其余所有患者临床状况良好。总之，儿科实践中的心脏肿瘤通常在临床和组织学上是良性的。只有少数病例需要手术。高达三分之一的心脏肿块在产前可被检测到。横纹肌瘤是最常见的组织学类型，其次是纤维瘤和黏液瘤。长期预后通常良好。
