儿童心脏肿瘤136例中长期随访

Mid - long term follow - up findings of cardiac tumors in 136 children

目的探讨儿童心脏肿瘤的病理类型、临床特点及自然转归，为选择合适的治疗方法提供依据。方法收集136例心脏肿瘤患儿历次心脏超声、心电图及心脏磁共振成像报告，查阅住院患儿病史、手术所见及组织病理结果。归类分析各种肿瘤的生长部位、相应临床表现、手术指征及中远期随访结果。结果136例儿童心脏肿瘤中原发性心脏肿瘤132例（97．1％），转移性心脏肿瘤4例（2．9％）；其中原发性心脏良性肿瘤126例（92．6％），最常见的是横纹肌瘤（66例），其次是纤维瘤（10例）、黏液瘤（5例）、血管瘤（5例）、脂肪瘤（3例）、乳头状纤维瘤（2例）、心包囊肿1例以及未分型34例（25．0％）。心脏恶性肿瘤10例（7．4％），其中原发性恶性肿瘤6例；转移性心脏肿瘤4例。手术切除肿瘤43例（31．6％），围手术期死亡4例（9．3％），远期死亡5例（11．7％）。横纹肌瘤中有22例行颅脑CT或磁共振成像检查，其中诊断为结节性硬化或高度怀疑结节性硬化17例（77．3％），横纹肌瘤有消退趋势者21例，占全部横纹肌瘤的31．8％。结论儿童心脏肿瘤以原发性良性肿瘤为主。心脏横纹肌瘤最多见，约三分之一患儿横纹肌瘤有消退趋势，如不伴有临床症状也无血流动力学影响，不必予以手术治疗。

Objective To explore the pathological type,clinical features and natural history of cardiac tumors in children, in order to provide the evidence for the selecting of appropriate treatment. Methods Totally 136 cases of children with cardiac tumors were enrolled. The data including the previous echocardiography, electrocardiogram and cardiac magnetic resonance imaging（MRI） reports,inpatient medical history, surgery findings and histopathological out- comes were investigated. The sites of various tumors, corresponding clinical manifestations, operative indications and the mid - long term follow - up findings were classified and analyzed on the basis of clinical data. Results There were 132 cases （97.1%） of primary cardiac tumors and 4 cases （2.9%） of secondary cardiac tumors in 136 cases of pediatric cardiac tumors; among the primary cardiac tumors, there were 126 patients （92.6%） with primary benign cardiac tumors,including rhabdomyoma 66 cases, fibroma 10 cases, myxoma 5 cases, hemangioma 5 cases, lipoma 3 cases, pa- pillary fibroelastoma 2 cases, pericardial cyst 1 case, and there were 34 cases undifferentiated. There were 6 patients with primary malignant cardiac tumors,4 cases with secondary cardiac tumors. Surgical procedures were conducted in 43 cases （31.6%） ,in which 4 patients （9.3%） died during perioperative period and 5 cases（ 11.7% ） died later. Among the 22 patients of rhabdomyoma performed brain computed tomography （CT） or MRI ,there were 17 cases diag- nosed or highly suspected tuberous sclerosis, accounting for 77.3% ;21 cases of rhabdomyoma had the trend of sponta- neously regress,accounting for 31.8% of all rhabdomyoma tumors. Conclusions Primary benign cardiac tumors were of high incidence in children. Rhabdomyoma is the most common cardiac tumor, about one - third of patients have the trend of spontaneously regress, so only when patients with hemodynamic compromise, heart failure, or associated with re- fractory rhythm disorders, surgical excision is required.