目的 总结儿童原发性心脏肿瘤的治疗效果。方法 收集2003年6月30日至2017年5月2日由本院确诊并行手术治疗的原发性心脏肿瘤患儿作为研究对象,回顾性分析患儿的年龄分布、临床表现、肿瘤部位、诊断、治疗、病理分型及预后等情况。结果 16例原发性心脏肿瘤患儿中,男童4例,女童12例; 年龄范围为1.3个月至12.5岁,平均年龄为2.45岁; 临床表现多以胸闷、气促为主; 14例为单发肿瘤,2例为多发肿瘤; 肿瘤位于左心房4例,左心室2例,右心房4例,右心室4例,室间隔2例; 患儿均行心脏彩超检查,其中8例行心脏MRI检查,1例行CTA检查; 12例心脏肿瘤完整切除,3例心脏肿瘤部分切除; 1例行心脏肿瘤活检术; 术后病理学诊断黏液瘤8例,纤维瘤3例,横纹肌瘤2例,婴儿型毛细血管瘤、脂肪瘤、脂肪母细胞瘤各1例。5例失访,11例随访3个月至6年,未出现肿瘤复发或肿瘤继续增长。结论 儿童原发性心脏肿瘤症状不典型,多在婴幼儿期诊断,心脏彩超是首选检查方法,部分需行心脏MRI检查,对有明显症状者应及时采取手术治疗,手术以恢复正常血流动力学状态为目的 ,可根据病变位置及大小选择行完整切除或部分切除,治疗效果良好。
Objective To summarize the experiences of diagnosing and treating primary cardiac tumors in children. Methods From 2003 to 2017,16 surgical cases of primary cardiac tumor were recruited and retrospective analyses performed for age distribution,clinical manifestations,tumor locations,diagnoses,treatment,pathologic classifications and prognoses. Results There were 4 boys and 12 girls aged from 1.3 months to 12.5 years.The major clinical manifestations included chest tightness and shortness of breath.In 14 cases,only one tumor were present while another other 2 cases had multiple tumors.Tumors were located in left atrium(n=4),left ventricle(n=2),right atrium(n=4),right ventricle(n=4)and ventricular septum(n=2).This group was examined by color echocardiography.Among them,8 patients received cardiac MRI examination while another routine computed tomography angiography(CTA).Resection was total(n=12)and partial(n=3).One case received cardiac tumor biopsy.The results of postoperative pathology examination were as follows: myxoma(n=8),fibroma(n=3),rhabdomyoma(n=2)and infantile capillary hemangioma,lipoma & lipoblastoma(n=1).Five cases were lost and 11 cases followed up for 3 months to 6 years.There was no recurrence or tumor growth. Conclusion Primary pediatric cardiac tumors have a low incidence and atypical symptoms.They are more frequently diagnosed in infants.Color echocardiography and MRI or CTA confirm a diagnosis.Those with obvious symptoms should be operated promptly for restoring normal hemodynamics.Complete resection or partial resection should be chosen according to the location and size of lesion.
