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小儿心脏肿瘤:一项为期45年的单机构回顾研究。

Pediatric cardiac tumors: a 45-year, single-institution review.

作者信息

Linnemeier Laura, Benneyworth Brian D, Turrentine Mark, Rodefeld Mark, Brown John

机构信息

Department of Cardiovascular Surgery, Indiana University School of Medicine, Indianapolis, IN, USA.

Department of Cardiovascular Surgery, Indiana University School of Medicine, Indianapolis, IN, USA Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.

出版信息

World J Pediatr Congenit Heart Surg. 2015 Apr;6(2):215-9. doi: 10.1177/2150135114563938.

DOI:10.1177/2150135114563938
PMID:25870340
Abstract

BACKGROUND

Cardiac tumors in children are rare. Of the cases reported in the literature, nearly all are benign and managed conservatively.

METHODS

This is a retrospective, observational study of pediatric patients <18 years who presented for surgical evaluation of a cardiac tumor, between 1969 and 2014 at a tertiary care children's hospital. Presentation, pathology, management, and outcomes were evaluated.

RESULTS

Over the last 45 years, 64 patients were evaluated for surgical resection of a cardiac tumor. Rhabdomyoma was the most common neoplasm (58%), and 17% of the tumors had malignant pathologies. While 42% of benign cardiac neoplasms required surgical intervention for significant hemodynamic concerns, 73% of malignant neoplasms underwent radical excision, if possible, followed by adjuvant chemotherapy. Despite a 37% mortality in patients with malignant pathology, an aggressive surgical approach can yield long-term survival in some patients. There were no deaths among patients with benign tumors and 17% had postoperative complications mostly related to mitral regurgitation.

CONCLUSION

Cardiac tumors in children are rare but can be managed aggressively with good outcomes. Benign tumors have an excellent survival with most complications related to tumor location. Malignant tumors have a high mortality rate, but surgery and adjuvant chemotherapy allow for prolonged survival in selected patients.

摘要

背景

儿童心脏肿瘤较为罕见。文献报道的病例中,几乎所有都是良性的,采用保守治疗。

方法

这是一项回顾性观察研究,研究对象为1969年至2014年期间在一家三级儿童专科医院接受心脏肿瘤手术评估的18岁以下儿科患者。对其临床表现、病理、治疗及预后进行评估。

结果

在过去45年里,64例患者接受了心脏肿瘤手术切除评估。横纹肌瘤是最常见的肿瘤(58%),17%的肿瘤为恶性病理类型。42%的良性心脏肿瘤因严重血流动力学问题需要手术干预,73%的恶性肿瘤尽可能接受根治性切除,随后进行辅助化疗。尽管恶性病理类型的患者死亡率为37%,但积极的手术方法可使部分患者长期存活。良性肿瘤患者无死亡病例,17%的患者术后出现并发症,主要与二尖瓣反流有关。

结论

儿童心脏肿瘤虽罕见,但可积极治疗并取得良好效果。良性肿瘤存活率高,多数并发症与肿瘤位置有关。恶性肿瘤死亡率高,但手术和辅助化疗可使部分患者延长生存期。

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