Rare primary cardiac myxofibrosarcoma in adolescence: Diagnostic challenges and surgical insights.

IF 2.3 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Cardiovascular Pathology Pub Date : 2024-02-17 DOI:10.1016/j.carpath.2024.107623
Jesús Machuca-Aguado , Rosa Rendón-García , Alessia Miraglia , Juan Carlos Téllez-Cantero , Álvaro Gutiérrez-Domingo
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Abstract

This article presents a unique case of primary myxofibrosarcoma, an exceptionally rare cardiac neoplasm, in a 16-year-old girl. With fewer than 40 documented cases, this tumor's occurrence in adolescence is particularly uncommon. The patient presented with right-sided paresthesia and subsequent right hemiparesis and dysarthria, along with bilateral pleural effusion. Imaging tests revealed a cerebral ischemic lesion and the presence of a left atrial mass. Initially suspected as a myxoma, the tumor exhibited significant infiltration. Despite prompt excision and cardiac intervention, the patient experienced rapid decompensation and succumbed to heart failure 2 days later. Histopathological analysis revealed an intermediate grade myxofibrosarcoma. This case underscores the diagnostic complexity, emphasizing the need for early identification given the grim prognosis associated with cardiac myxofibrosarcomas, requiring detailed imaging, surgical, and histopathological insights.

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青少年罕见的原发性心脏肌纤维肉瘤:诊断难题与手术启示。
本文介绍了一例独特的原发性肌纤维肉瘤病例,这是一种非常罕见的心脏肿瘤,患者是一名16岁的女孩。有记录的病例不到 40 例,这种肿瘤发生在青少年时期尤为罕见。患者出现右侧麻痹,随后出现右侧偏瘫和构音障碍,并伴有双侧胸腔积液。影像学检查发现其脑部有缺血性病变,左心房有肿块。最初怀疑是肌瘤,但肿瘤有明显浸润。尽管及时切除了肿瘤并进行了心脏介入治疗,但患者病情迅速恶化,两天后死于心力衰竭。组织病理学分析显示,这是一种中度肌纤维肉瘤。该病例凸显了诊断的复杂性,强调鉴于心脏肌纤维肉瘤的预后很差,需要详细的影像学、外科手术和组织病理学检查才能早期发现。
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来源期刊
Cardiovascular Pathology
Cardiovascular Pathology 医学-病理学
CiteScore
7.50
自引率
2.70%
发文量
71
审稿时长
18 days
期刊介绍: Cardiovascular Pathology is a bimonthly journal that presents articles on topics covering the entire spectrum of cardiovascular disease. The Journal''s primary objective is to publish papers on disease-oriented morphology and pathogenesis from clinicians and scientists in the cardiovascular field. Subjects covered include cardiovascular biology, prosthetic devices, molecular biology and experimental models of cardiovascular disease.
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