侵袭性心脏黏液纤维肉瘤进展迅速1例报告。

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
European Heart Journal: Case Reports Pub Date : 2025-09-16 eCollection Date: 2025-10-01 DOI:10.1093/ehjcr/ytaf447
Koichi Nakamura, Osamu Kurihara, Nobuaki Kobayashi, Yasuhiro Kawase, Kuniya Asai
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引用次数: 0

摘要

背景:原发性心脏黏液纤维肉瘤是一种非常罕见的恶性肿瘤,通常无症状,直到局部侵袭或远处转移发生。早期诊断具有挑战性,由于局部复发和转移率高,预后通常较差。病例总结:我们报告一例79岁的女性,她表现出神经系统症状和多发性脑转移。影像学显示一个广泛的外生肿块阻塞了左肺静脉并延伸到左心房。手术切除,病理证实为黏液纤维肉瘤。尽管进行了手术,肿瘤在几天内局部复发,并且脑转移进展。考虑到患者病情恶化,未开始进一步治疗。随后,她被转移到临终关怀医院,不久就去世了。讨论:原发性心脏黏液纤维肉瘤是一种极其罕见的恶性肿瘤,由于其侵袭性的生物学行为,其症状无特异性,预后差。本例多模态影像显示提示恶性肿瘤的特征,包括外生性肿块,附着基础广泛,信号强度不均。尽管手术切除,肿瘤复发迅速,脑转移进展。肿瘤大小、高组织学分级和延迟干预可能导致临床结果不佳。由于其罕见性,对于原发性心脏肉瘤没有标准化的治疗指南。在这个病例中,我们通过在短时间内进行的多次计算机断层扫描再次确认了心脏肉瘤的高度恶性。目前,使用多种影像方式进行及时评估的唯一方法是早期手术切除联合辅助治疗,以改善临床结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Case report of an aggressive cardiac myxofibrosarcoma with rapid disease progression.

Background: Primary cardiac myxofibrosarcoma is an exceptionally rare malignant tumour, often asymptomatic until local invasion or distant metastasis occurs. Early diagnosis is challenging, and the prognosis is generally poor owing to high local recurrence and metastasis rates.

Case summary: We report the case of a 79-year-old woman who presented with neurological symptoms and multiple brain metastases. Imaging showed a broad-based exophytic mass obstructing the left pulmonary vein and extending into the left atrium. Surgical resection was conducted, and the diagnosis of myxofibrosarcoma was confirmed from histology. Despite surgery, the tumour recurred locally within days, and the brain metastases progressed. Considering the patient's deteriorating condition, no further treatment was initiated. She was then transferred to hospice care and died shortly thereafter.

Discussion: Primary cardiac myxofibrosarcoma is an extremely rare malignancy with non-specific symptoms and poor prognosis owing to its aggressive biological behaviour. In this case, multimodal imaging showed features suggestive of malignancy, including an exophytic mass with a broad attachment base and heterogeneous signal intensity. Despite surgical resection, the tumour recurred rapidly, and brain metastases progressed. The tumour size, high histological grade, and delayed intervention are likely to contribute to poor clinical outcomes. Owing to their rarity, there are no standardized treatment guidelines for primary cardiac sarcomas. In this case, we reconfirmed the high-grade malignancy of the cardiac sarcoma through multiple computerized tomography scans conducted over a short period. Currently, the only method for prompt evaluation using multiple imaging modalities involves early surgical resection combined with adjuvant therapy to improve clinical outcomes.

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来源期刊
European Heart Journal: Case Reports
European Heart Journal: Case Reports Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.30
自引率
10.00%
发文量
451
审稿时长
14 weeks
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