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有症状的胎儿型心脏横纹肌瘤

Symptomatic Fetal-Type Cardiac Rhabdomyoma.

作者信息

Kayali Seyma, Dogan Vehbi, Arda Nilüfer, Koç Murat, Ertugrul Ilker, Özgür Senem, Örün Utku Arman, Karademir Selmin

机构信息

Department of Pediatric Cardiology, Dr. Sami Ulus Maternity and Children Research and Training Hospital, Pediatric Cardiology, Ankara, Turkey.

Department of Pathology, Dr. Sami Ulus Maternity and Children Research and Training Hospital, Pediatric Cardiology, Ankara, Turkey.

出版信息

J Coll Physicians Surg Pak. 2017 Mar;27(3):S53-S55.

Abstract

Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

摘要

横纹肌瘤是最常见的原发性心脏肿瘤,尤其在儿童早期可见。胎儿型横纹肌瘤是一种几乎总是在心脏外部位发现的良性肿瘤。尽管心脏横纹肌瘤的自然病程是消退,但胎儿型横纹肌瘤在心脏中出现时的行为因其罕见性而尚不明确。在此,我们报告一例血流动力学不稳定的女性新生儿,其心脏内有一个单一的大肿块,药物治疗无效,接受了手术。该新生儿因心室颤动手术未能存活。尸检时肿块被诊断为胎儿型心脏横纹肌瘤。

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