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一例手术切除的伴有进行性左心室流出道梗阻的心脏横纹肌瘤。

A case of surgically resected cardiac rhabdomyoma with progressive left ventricular outflow tract obstruction.

机构信息

Department of Laboratory Diagnostic Services, Universiti Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia.

Department of Pathology, Universiti Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia; Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Columbus OH 43205, USA.

出版信息

Cardiovasc Pathol. 2020 Nov-Dec;49:107226. doi: 10.1016/j.carpath.2020.107226. Epub 2020 May 12.

Abstract

Cardiac rhabdomyoma is the most prevalent cardiac tumors in the pediatric population, in close association with tuberous sclerosis complex. It is usually detected antenatally or postnatally by echocardiography. Clinical presentations depend greatly on the size and position of the tumor mass. Interestingly, rhabdomyoma has a propensity to regress spontaneously and is not usually operated upon, unless the patient becomes hemodynamically compromised. Herein, we report an unusual case of surgically treated cardiac rhabdomyoma in a baby boy presented at birth with a progressive enlarging intraventricular mass, complicated with left ventricular outflow tract obstruction 7 weeks later. Histopathological examination of the intracardiac mass revealed sheets of tumor cells with spider-like morphology (known as "spider cells"), confirmed the diagnosis of rhabdomyoma. Close disease monitoring of patient's hemodynamic status in a newly diagnosed cardiac rhabdomyoma is inevitable as the tumor, although rare, may progress.

摘要

心脏横纹肌瘤是儿科人群中最常见的心脏肿瘤,与结节性硬化症密切相关。它通常通过超声心动图在产前或产后被检测到。临床表现很大程度上取决于肿瘤的大小和位置。有趣的是,横纹肌瘤有自发消退的倾向,通常不需要手术,除非患者出现血液动力学障碍。在此,我们报告一例婴儿心脏横纹肌瘤的罕见病例,该婴儿出生时即表现为进行性增大的室内肿块,7 周后并发左心室流出道梗阻。心脏肿块的组织病理学检查显示出片状的肿瘤细胞,具有蜘蛛样形态(称为“蜘蛛细胞”),这证实了横纹肌瘤的诊断。新诊断的心脏横纹肌瘤患者的血液动力学状态的密切疾病监测是不可避免的,因为尽管罕见,但肿瘤可能会进展。

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