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[表现为右心房肿块的血管内平滑肌瘤病]

[Intravascular leiomyomatosis presenting as a right atrial mass].

作者信息

Magyar Eva, Salamon Ferenc, Tomcsányi István

机构信息

Országos Gyógyintézeti Központ, Patológiai Osztály, H-1031 Budapest.

出版信息

Lege Artis Med. 2007 Oct;17(10):694-7.

Abstract

INTRODUCTION

Intravenous leiomyoma is a peculiar, rare tumour that originates from the myomatous uterus, grows into the lumina of veins and, through the inferior vena cava, may extend to the right heart cavity. The intracardial tumour causes severe cardiovascular symptoms and may be fatal.

CASE REPORT

The symptoms of the 45-year-old woman consisted of chest discomfort, dizziness, and short periods of unconsciousness, which occurred during vacuum-cleaning when she bent forward. Imaging showed a growth that filled the lumen of the vena cava and extended to the right atrium, swinging between the atrium and ventricle. The patient underwent surgery with cardiopulmonary bypass and the intracardial tumour was removed together with its extensions filling the veins. The histological examination verified intravenous leiomyomatosis. Although the patient refused the recommended hysterectomy, she is well after six years the operation.

CONCLUSION

In cases of unspecific cardiac symptoms, the possibility of intravenous leiomyomatosis should be considered. The diagnosis can be set up by echocardiography, MR or CT scan. If the diagnosis is confirmed, the tumour should be surgically removed.

摘要

引言

静脉内平滑肌瘤是一种特殊的罕见肿瘤,起源于子宫肌瘤,生长进入静脉腔,并可通过下腔静脉延伸至右心腔。心内肿瘤会引起严重的心血管症状,可能致命。

病例报告

这位45岁女性的症状包括胸部不适、头晕以及在弯腰吸尘时出现的短暂意识丧失。影像学检查显示有一个肿物充满了腔静脉腔并延伸至右心房,在心房和心室之间摆动。患者接受了体外循环手术,心内肿瘤及其充满静脉的延伸部分被一并切除。组织学检查证实为静脉内平滑肌瘤病。尽管患者拒绝了建议的子宫切除术,但术后六年情况良好。

结论

对于出现非特异性心脏症状的病例,应考虑静脉内平滑肌瘤病的可能性。可通过超声心动图、磁共振成像或计算机断层扫描进行诊断。如果诊断得到证实,应手术切除肿瘤。

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