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左心房原发性未分化高级多形性心脏肉瘤,表现不典型。

Primary undifferentiated high-grade pleomorphic cardiac sarcoma in the left atrium with atypical presentation.

作者信息

Tan Sue Fen, Abbas Sherif Osama Ahmed, Mayooran Nithiananthan, Naik Surendra Kashinath

机构信息

Cardiac surgery, Nottingham University Hospitals NHS Trust, Nottingham, UK

Cardiac surgery, Nottingham University Hospitals NHS Trust, Nottingham, UK.

出版信息

BMJ Case Rep. 2023 Jan 23;16(1):e252198. doi: 10.1136/bcr-2022-252198.

Abstract

A woman in her 60s had 4 months of malaise, fatigue, dyspnoea, night sweats and grade 3 clubbing. She had a pansystolic murmur and signs of congestive heart failure. Multimodal imaging with a transthoracic echocardiogram, transoesophageal echocardiogram and CT was carried out to diagnose and reconstruct the mass for surgical planning. A 2×2.8 cm circular dense mass was found in the left atrium with vegetations on the mitral valve. Positron emission tomography and cardiac MRI confirmed the mass's hypermetabolic activity and malignant features. Mitral valve replacement and surgical resection were performed. She was diagnosed with grade 3 undifferentiated cardiac sarcoma and referred to the sarcoma multidisciplinary team for further management given sarcoma's rarity and complexity. The poor prognosis of sarcoma was evident as her symptoms recurred 2 months postoperatively.

摘要

一位 60 多岁的女性出现了 4 个月的不适、疲劳、呼吸困难、盗汗和 3 级杵状指。她有全收缩期杂音和充血性心力衰竭的体征。通过经胸超声心动图、经食管超声心动图和 CT 进行多模态成像,以进行诊断和重建肿块用于手术计划。在左心房发现一个 2×2.8 厘米的圆形致密肿块,二尖瓣上有赘生物。正电子发射断层扫描和心脏 MRI 证实了肿块的高代谢活性和恶性特征。进行了二尖瓣置换和手术切除。由于肉瘤的罕见性和复杂性,她被诊断为 3 级未分化心脏肉瘤,并转介给肉瘤多学科团队进行进一步管理。肉瘤的预后很差,因为她在手术后 2 个月症状复发。

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