目的

回顾性分析儿童原发性心脏肿瘤（PCT）的超声表现及预后。

方法

回顾性收集2017 年3 月至2024 年5 月由西安交通大学附属儿童医院确诊并经手术病理证实的儿童PCT 患儿15 例，分析患儿基本信息、临床及超声特点，肿瘤位置、形态、大小、活动度、切除方式、病理及预后等情况。

结果

15 例患儿中，心脏良性肿瘤12 例（80.0%），包括横纹肌瘤6 例，纤维瘤2 例，黏液瘤2 例，毛细血管瘤1 例，脂肪瘤1 例；中间型肿瘤2 例（13.3%），均为炎性肌纤维母细胞瘤；心脏恶性肿瘤1 例（6.7%），为CIC 重排肉瘤。横纹肌瘤患儿年龄、体质量及瘤体最大径均小于其他病理类型。15 例患儿中，单发肿瘤12 例，多发肿瘤3 例（多发横纹肌瘤2 例，多发黏液瘤1 例），瘤体数量共计22 个，位于左心室15 个，右心室4 个，左心房1 个，右心房1 个，心包1 个。合并心包积液者3 例。术前超声与病理诊断一致者9 例（60.0%），误诊4 例（2 例单发横纹肌瘤，因随心动周期轻微摆动，误诊为黏液瘤；1 例可轻微摆动的带蒂毛细血管瘤误诊为黏液瘤；1 例伴钙化的炎性肌纤维母细胞瘤误诊为黏液瘤），诊断不明2 例。15 例患儿中，3 例行瘤体部分切除，12 例行瘤体完全清除，围手术期无死亡病例。1 例心脏恶性肿瘤患儿术后5 个月死亡，余14 例患儿术后心功能良好，随访6 个月～7 年均未出现肿瘤复发或二次手术。

结论

儿童PCT 以良性肿瘤为主，部分PCT超声表现具有特异性；少见类型肿瘤术前诊断较困难，确诊需依赖病理学检查。良性及中间型PCT手术治疗预后良好。

Objective

To retrospectively analyze the ultrasonographic characteristics and outcomes of primary cardiac tumors (PCTs) in children.

Methods

A total of 15 infants or children with PCTs who underwent surgery and pathology examination at Children’s Hospital Affiliated to Xi ’an Jiaotong University from March 2017 to May 2024 were retrospectively included in this study.Gender, clinical symptoms, and echocardiographic results, as well as tumor location, size, pathology, and prognosis, were analyzed.

Results

Among the 15 pediatric patients included, 12 (80.0%) had benign cardiac tumors (rhabdomyoma in 6 cases,fibroma in 2, myxoma in 2, hemangioma in 1, and lipoma in 1), 2 (13.3%) had intermediate-type tumors(inflammatory myofibroblastoma in both), and 1 (6.7%) had malignant cardiac tumor (CIC rearrangement sarcoma).Patients with rhabdomyoma exhibited lower age, body weight, and maximum tumor diameter compared to other pathological subtypes.Tumor distribution analysis showed solitary lesions in 12 cases and multiple lesions in 3.In total, there were 22 lesions, and the locations were as follows: 15 lesions in the left ventricle, 4 in the right ventricle, 1 in the left atrium, 1 in the right atrium, and 1 in the pericardium.Concomitant pericardial effusion was found in 3 cases.Preoperative echocardiography findings were coincident with pathological diagnoses in 9 cases (60.0%), with 4 misdiagnoses (2 solitary rhabdomyomas misdiagnosed as myxomas; 1 pedunculated capillary hemangioma misdiagnosed as myxoma; 1 inflammatory myofibroblastic tumor misdiagnosed as myxoma), and 2 cases remained undiagnosed.Partial tumor resection was performed in 3 cases, and complete resection in 12, with no perioperative mortality.The patient with malignant cardiac tumor died 5 months after operation, and the other 14 cases were alive, with no tumor recurrence or reoperation during 0.5 to 10 years of follow-up.

Conclusion

The majority of PCTs in children are benign, some of them have specific echocardiographic manifestations, and rare types need pathological examination for diagnosis.The prognosis of benign and intermediate PCTs in children undergoing surgical resection is satisfactory.