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小儿年龄组原发性心脏肿瘤:对临床医生相关显著病理特征的综述

Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians.

作者信息

Becker A E

机构信息

Department of Cardiovascular Pathology, Academic Medical Center, University of Amsterdam, Post Office Box 22700, 1100 DE Amsterdam, The Netherlands.

出版信息

Pediatr Cardiol. 2000 Jul-Aug;21(4):317-23. doi: 10.1007/s002460010071.

DOI:10.1007/s002460010071
PMID:10865004
Abstract

Because primary tumors of the heart in infants and children are extremely rare, most knowledge is based on collections of case reports rather than large cohort studies. The types of heart tumors encountered in the pediatric age group differ from those seen in adults. In the latter, cardiac myxomas are by far the most common tumor; in infants and children the most common primary tumor of the heart is the rhabdomyoma. Spontaneous regression of these tumors has been well established so that surgical intervention is no longer indicated unless there are clinical manifestations from the heart. There is a high incidence of associated tuberous sclerosis. Any intracavitary mass in infants is suggestive for a cardiac rhabdomyoma unless otherwise proven. The second most common tumor in this age group is cardiac fibroma. These tumors probably represent hamartomatous lesions and this has led to a strategy in which radical surgical excision is not indicated if the procedure endangers postoperative heart function. Cardiac myxomas are of interest in this age group because of their familial occurrence as part of the myxoma syndrome. Other types of primary heart tumors occur, including malignant variants, but all are exceedingly rare. It is because of the sporadic nature of these tumors that treatment and insights into prognosis are based largely on case documentations and analogies from similar tumors originating elsewhere.

摘要

由于婴幼儿心脏原发性肿瘤极为罕见,多数认知基于病例报告的汇总,而非大型队列研究。儿童年龄组中所遇到的心脏肿瘤类型与成人不同。在成人中,心脏黏液瘤是迄今为止最常见的肿瘤;而在婴幼儿和儿童中,最常见的心脏原发性肿瘤是横纹肌瘤。这些肿瘤的自发消退已得到充分证实,因此除非有心脏的临床表现,否则不再需要手术干预。结节性硬化症的伴发率很高。除非另有证实,否则婴儿的任何心腔内肿块都提示为心脏横纹肌瘤。该年龄组中第二常见的肿瘤是心脏纤维瘤。这些肿瘤可能代表错构瘤性病变,这导致了一种策略,即如果手术危及术后心脏功能,则不建议进行根治性手术切除。心脏黏液瘤在该年龄组中受到关注是因为它们作为黏液瘤综合征的一部分具有家族性发生情况。其他类型的原发性心脏肿瘤也会出现,包括恶性变体,但都极为罕见。正是由于这些肿瘤的散发性,治疗方法和对预后的认识在很大程度上基于病例记录以及源自其他部位的类似肿瘤的类推。

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