Shi Lin, Wu Lanping, Fang Huijuan, Han Bo, Yang Jialun, Ma Xiaojin, Liu Fang, Zhang Yongwei, Xiao Tingting, Huang Min, Huang Meirong
Shanghai Children's Medical Center, Medical Institute, Shanghai Jiaotong University, Shanghai, China.
, Shandong Provincial Hospital, Jinan, People's Republic of China.
Eur J Pediatr. 2017 Feb;176(2):253-260. doi: 10.1007/s00431-016-2833-4. Epub 2017 Jan 10.
The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors. Among the 158 cases of primary cardiac tumor, 150 were benign and eight were malignant. The rhabdomyoma, fibroma, and myxoma are the most common types of benign cardiac tumors. The major clinical manifestations of cardiac tumors include outflow tract obstruction, arrhythmia, dyspnea, pericardial effusion, heart failure, and seizures. Among the 59 patients who underwent surgery, 49 had primary benign cardiac tumors, eight had primary malignant tumors, and two had malignant metastatic tumors. Post-surgery, nine of the patients had residual tumor tissues that did not significantly affect their hemodynamics. Following surgery, there were two cases of recurrence and nine deaths, including four of benign and five of malignant tumors with mortality rates of 8.2 and 50.0 %, respectively. Of the remaining 107 cases of patients who did not undergo surgery, five (4.7 %) died.
The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors. What is known: • Pediatric cardiac tumors are rare and are predominantly primary and benign. • The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors. What is new: • The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors. • If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.
本研究旨在探讨儿童心脏肿瘤的病理分类、临床特征和自然病史,为选择合适的治疗方法提供依据。对四家医院心脏肿瘤门诊或住院患者的病历进行分类,分析各种类型肿瘤的生长部位、临床表现、手术指征和长期随访结果。共有166例患者,其中原发性心脏肿瘤158例,转移性心脏肿瘤6例,未分类心脏肿瘤2例。在158例原发性心脏肿瘤中,150例为良性,8例为恶性。横纹肌瘤、纤维瘤和黏液瘤是最常见的良性心脏肿瘤类型。心脏肿瘤的主要临床表现包括流出道梗阻、心律失常、呼吸困难、心包积液、心力衰竭和惊厥。在接受手术的59例患者中,49例为原发性良性心脏肿瘤,8例为原发性恶性肿瘤,2例为恶性转移性肿瘤。术后,9例患者有残留肿瘤组织,但对其血流动力学无明显影响。手术后,有2例复发和9例死亡,其中良性肿瘤4例,恶性肿瘤5例,死亡率分别为8.2%和50.0%。其余107例未接受手术的患者中,5例(4.7%)死亡。
原发性良性心脏肿瘤是儿童心脏肿瘤的主要类型,其中横纹肌瘤、纤维瘤和黏液瘤是最常见的类型。如果没有严重症状且血流动力学未受影响,大多数患者尽管患有肿瘤仍可长期存活。已知信息:• 儿童心脏肿瘤罕见,主要为原发性和良性。• 心力衰竭、心律失常和流出道梗阻症状是心脏肿瘤最严重的并发症。新发现:• 横纹肌瘤、纤维瘤和黏液瘤是原发性良性心脏肿瘤最常见的类型。• 如果没有严重症状且血流动力学未受影响,大多数患者尽管患有肿瘤仍可长期存活。