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一名年轻女性患者患有无症状性右心房平滑肌肉瘤并伴有三尖瓣梗阻。

Asymptomatic right atrial leiomyosarcoma with tricuspid valve obstruction in a young female patient.

作者信息

Kornberg A, Wildhirt S M, Kreuzer E, Reichart B

机构信息

Department of Cardiac Surgery, Ludwig-Maximilian-University, Munich, Klinikum Grosshadern, Germany.

出版信息

Eur J Cardiothorac Surg. 1998 Dec;14(6):635-8. doi: 10.1016/s1010-7940(98)00243-7.

Abstract

Primary cardiac leiomyosarcomas of the heart are rare tumors usually diagnosed post-mortem. Like other sarcomas located in the heart, it causes symptoms by obstruction or occlusion of cardiac cavities, local invasion, embolization or by systemic manifestations. We present an unusual case of a previously healthy young female patient who was accidentally diagnosed with a cardiac tumor of unknown origin during routine physical examination in May 1997. Until a few days prior to elective cardiac surgery for diagnostic purposes in June 1997, no clinical symptoms were present. To our surprise, a primary right atrial leiomyosarcoma was found which almost completely occluded the right atrium and destroyed the tricuspid valve. Despite the combination of surgical removal and adjuvant chemotherapy the patient died 3 weeks after the operation due to progressive tumor disease and development of congestive heart failure and lung embolism.

摘要

原发性心脏平滑肌肉瘤是一种罕见的肿瘤,通常在尸检时才被诊断出来。与位于心脏的其他肉瘤一样,它通过阻塞或闭塞心腔、局部侵犯、栓塞或全身表现引起症状。我们报告一例不寻常的病例,一名既往健康的年轻女性患者,于1997年5月例行体检时意外诊断出起源不明的心脏肿瘤。在1997年6月为明确诊断而进行择期心脏手术的前几天,患者均无临床症状。令人惊讶的是,术中发现是原发性右房平滑肌肉瘤,几乎完全阻塞了右心房并破坏了三尖瓣。尽管进行了手术切除并辅助化疗,但患者术后3周因肿瘤进展、充血性心力衰竭和肺栓塞而死亡。

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