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首例转移性黏液样脂肪肉瘤侵犯左心房腔及肺静脉的病例报告。

The first case report of a metastatic myxoid liposarcoma invading the left atrial cavity and pulmonary vein.

作者信息

Dogan Umuttan, Zamani Adil, Gormus Niyazi, Paksoy Yahya, Avunduk Mustafa Cihat, Demirbas Soner

机构信息

Department of Cardiology, Selcuk University, Meram School of Medicine, Konya, Turkey.

出版信息

Heart Surg Forum. 2011 Aug;14(4):E261-3. doi: 10.1532/HSF98.20111021.

Abstract

Myxoid liposarcoma (MLS) is the most commonly encountered liposarcoma subgroup, accounting for about 50% of all cases. Metastatic MLS of the heart is extremely rare. Herein we describe for the first time metastasis of MLS to the left atrium and left upper pulmonary vein in a 54-year-old woman who was admitted with shortness of breath and cough persisting for 2 weeks. The patient reported that a total surgical excision of MLS of the left thigh followed by radiotherapy was performed 4 years ago. An emergency operation was performed due to rapidly progressive worsening of clinical condition and echocardiographic determination of left atrial mass protruding into the left ventricle and obstructing the mitral inflow throughout the diastole. The mass could not be totally excised because it was tightly adhered to the surrounding tissue. Postoperative magnetic resonance imaging (MRI) showed a 5 × 3 cm residual tumor deforming the posterior wall of the left atrium entirely and extending into the left upper pulmonary vein. Histopathological examination was consistent with MLS. In conclusion, considering probable cardiac metastasis in patients presenting with respiratory symptoms with medical history of soft tissue sarcomas would be life saving. The case is discussed, and a review of the literature in relation to the metastatic involvement of the heart by MLS is presented.

摘要

黏液样脂肪肉瘤(MLS)是最常见的脂肪肉瘤亚型,约占所有病例的50%。心脏转移性MLS极为罕见。在此,我们首次描述了一名54岁女性患者,因持续2周的呼吸急促和咳嗽入院,其MLS转移至左心房和左上肺静脉。患者报告4年前对左大腿的MLS进行了手术全切,随后进行了放疗。由于临床状况迅速恶化,且超声心动图确定左心房肿块突入左心室并在整个舒张期阻塞二尖瓣血流,遂进行了急诊手术。由于肿块与周围组织紧密粘连,无法完全切除。术后磁共振成像(MRI)显示一个5×3 cm的残留肿瘤,使左心房后壁完全变形并延伸至左上肺静脉。组织病理学检查与MLS相符。总之,对于有软组织肉瘤病史且出现呼吸道症状的患者,考虑可能的心脏转移可能会挽救生命。本文对该病例进行了讨论,并对MLS心脏转移累及的相关文献进行了综述。

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