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一例复杂的心脏内平滑肌瘤病临床病例。

A complex clinical case of intracardiac leiomyomatosis.

作者信息

Li Yuanzhi, Li Yidan, Liang Min, Sun Lanlan, Zhu Weiwei, Wei Liqun, Ding Xueyan, Guo Dichen, Lv Xiuzhang

机构信息

Department of Ultrasound, Beijing Chao Yang Hospital, Capital Medical University, No. 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, 100020, China.

Department of Radiology, Beijing Chao Yang Hospital, Capital Medical University, No. 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, 100020, China.

出版信息

BMC Cardiovasc Disord. 2025 Feb 17;25(1):105. doi: 10.1186/s12872-025-04561-7.

Abstract

BACKGROUND

Intracardiac leiomyomatosis (ICL) is an uncommon condition characterized by the proliferation of intravascular tissue within the veins, leading to the development of tumor emboli. This can pose a significant threat to life when the tumor invades cardiac structures. The diagnostic process for this condition is complex and presents considerable challenges.

CASE PRESENTATION

We report a case of a 38-year-old female patient whose pulmonary artery computed tomography (CT) revealed low density structure in the branches of the pulmonary artery. Echocardiography revealed a mobile tumor within the right heart chambers and pulmonary trunks as well as characteristic thickening of the ventricular septum consistent with hypertrophic cardiomyopathy (HCM). Magnetic resonance imaging (MRI) revealed a mass in the right anterior uterine wall, extending to the inferior vena cava (IVC) and right iliac vein. Post-surgery histopathological analysis confirmed a diagnosis of intravenous leiomyomatosis (IVL).

CONCLUSIONS

When IVL affects the heart, echocardiography is the best diagnostic tool for detecting the disease. CT and MRI are essential in identifying the location and extent of the tumor, as well as in evaluating prognosis.

摘要

背景

心脏内平滑肌瘤病(ICL)是一种罕见疾病,其特征为静脉内血管组织增生,导致肿瘤栓子形成。当肿瘤侵犯心脏结构时,会对生命构成重大威胁。这种疾病的诊断过程复杂,存在相当大的挑战。

病例报告

我们报告一例38岁女性患者,其肺动脉计算机断层扫描(CT)显示肺动脉分支内有低密度结构。超声心动图显示右心腔和肺动脉主干内有一个可移动的肿瘤,以及与肥厚型心肌病(HCM)一致的室间隔特征性增厚。磁共振成像(MRI)显示右前子宫壁有一个肿块,延伸至下腔静脉(IVC)和右髂静脉。术后组织病理学分析确诊为静脉内平滑肌瘤病(IVL)。

结论

当IVL累及心脏时,超声心动图是检测该疾病的最佳诊断工具。CT和MRI对于确定肿瘤的位置和范围以及评估预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a1c/11831790/d5291d793320/12872_2025_4561_Fig1_HTML.jpg

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