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室间隔心脏炎性肌纤维母细胞瘤:一例罕见病例报告。

Cardiac inflammatory myofibroblastic tumor in interventricular septum: A rare case report.

作者信息

Deng Ming-Dan, Han Jun-Yang, Lin Ke, Tang Hong

机构信息

Department of Cardiology, West China Hospital, Sichuan University.

Department of Cardiovascular Surgery, West China, Hospital of Sichuan University, Guoxue Xiang 37, Chengdu, Sichuan, PR China.

出版信息

Medicine (Baltimore). 2018 Nov;97(48):e13219. doi: 10.1097/MD.0000000000013219.

Abstract

RATIONALE

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare primary cardiac tumor which is currently considered as a low-grade neoplasm. The tumor has a predilection in infants and adolescents and primarily occurs as an endocardial-based cavitary mass. However, cardiac IMT that only involves the interventricular septum in middle-aged adults is extremely rarely reported. Considering its infrequency, we report a rare clinical case, with the aim of sharing our experience during the diagnostic procedures.

PATIENT CONCERNS

A 45-year-old, previously healthy female, with no medical history was admitted to the outpatient clinic due to the identification of an abnormal radiographic finding during a routine health examination.

DIAGNOSIS

Transthoracic echocardiography (TTE) revealed a 3.5 cm × 4.0 cm × 4.5 cm heterogeneous mass in the interventricular septum. Color Doppler echocardiography detected sparse blood flow signals inside the mass. Magnetic resonance imaging (MRI) confirmed a hyperintense T2-weighted, isointense T1-weighted mass. Three-dimensional (3D) TTE demonstrated a spherical mass in the middle part of the interventricular septum. Postoperative histopathological examinations revealed a mesenchymal tumor composed of scattered spindle myofibroblasts with a myxoid atypia, associated with infiltration of lymphocytes and plasma cells.

INTERVENTIONS

Complete tumor resection was successfully performed via median sternotomy under general anesthesia.

OUTCOMES

After surgery, the patient recovered successfully. The patient was in good general health without any clinical symptoms. The echocardiographic examination at the 12-month follow-up period revealed normal function of the heart, and there was no evidence of tumor recurrence.

LESSONS

To our knowledge, cardiac IMT only the involving interventricular septum in a middle-aged adult has never been previously reported before. Echocardiography plays a critical role in establishing the primary diagnosis of cardiac IMT and evaluating regular follow-up examinations. Complete surgical resection of the mass is considered the first-line treatment despite the absence of symptoms.

摘要

原理

心脏炎性肌纤维母细胞瘤(IMT)是一种罕见的原发性心脏肿瘤,目前被认为是一种低级别肿瘤。该肿瘤好发于婴幼儿和青少年,主要表现为心内膜下的空洞性肿块。然而,仅累及中年成人室间隔的心脏IMT极为罕见。鉴于其罕见性,我们报告一例罕见的临床病例,旨在分享诊断过程中的经验。

患者情况

一名45岁、既往健康、无病史的女性因在常规健康检查中发现异常影像学表现而入住门诊。

诊断

经胸超声心动图(TTE)显示室间隔有一个3.5厘米×4.0厘米×4.5厘米的不均匀肿块。彩色多普勒超声心动图检测到肿块内有稀疏血流信号。磁共振成像(MRI)证实为T2加权像高信号、T1加权像等信号的肿块。三维(3D)TTE显示室间隔中部有一个球形肿块。术后组织病理学检查显示为间叶性肿瘤,由散在的梭形肌纤维母细胞组成,伴有黏液样异型性,伴有淋巴细胞和浆细胞浸润。

干预措施

在全身麻醉下通过正中胸骨切开术成功进行了肿瘤完整切除。

结果

手术后,患者顺利康复。患者全身状况良好,无任何临床症状。术后12个月的超声心动图检查显示心脏功能正常,无肿瘤复发迹象。

经验教训

据我们所知,此前从未有过仅累及中年成人室间隔的心脏IMT的报道。超声心动图在心脏IMT的初步诊断及评估定期随访检查中起着关键作用。尽管无症状,肿块的完整手术切除仍被视为一线治疗方法。

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