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A case of surgically resected cardiac rhabdomyoma with progressive left ventricular outflow tract obstruction.
Cardiovascular Pathology ( IF 3.7 ) Pub Date : 2020-05-12 , DOI: 10.1016/j.carpath.2020.107226
Fazarina Mohammed 1 , Geok Chin Tan 2 , Kan N Hor 3 , Michael Arnold 4 , Yin Ping Wong 5
Affiliation  

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.



中文翻译:

一例手术切除的心脏横纹肌瘤伴进行性左心室流出道梗阻。

心脏横纹肌瘤是小儿人群中最普遍的心脏肿瘤,与结节性硬化症密切相关。通常在产前或产后通过超声心动图检查。临床表现在很大程度上取决于肿瘤块的大小和位置。有趣的是,横纹肌瘤具有自发性消退的倾向,并且除非患者血液动力学受到损害,否则通常不会对其进行手术。本文中,我们报道了一个男婴出生时出现外科横纹肌瘤的不寻常病例,该男婴出生时进展性脑室内肿块增大,并在7周后并发左心室流出道梗阻。对心内包块的组织病理学检查显示出具有蜘蛛状形态的肿瘤细胞片(称为“蜘蛛细胞”),确诊为横纹肌瘤。在新诊断的心脏横纹肌瘤中,对患者血液动力学状况进行密切的疾病监测是不可避免的,因为这种肿瘤尽管罕见,但可能会进展。

更新日期:2020-05-12
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