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纵隔淋巴管平滑肌瘤病:一例报告并文献复习

Mediastinal lymphangioleiomyomatosis: a case report and literature review.

作者信息

Wang Yike, Wang Quanyong, Shi Lin, Zhao Lei, Dou Yana, Hao Fene

机构信息

Department of Radiology, Affiliated Hospital of Inner Mongolia Medical University, Huhhot, Inner Mongolia, China.

Department of Radiology, Inner Mongolia Autonomous Region Hospital of Traditional Chinese Medicine, Huhhot, Inner Mongolia, China.

出版信息

Front Oncol. 2025 Sep 9;15:1588165. doi: 10.3389/fonc.2025.1588165. eCollection 2025.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disorder that primarily affects women of childbearing age. It is characterized by the abnormal growth of smooth muscle-like cells. While LAM typically occurs in the lungs, it can also be found in the retroperitoneum and pelvis. However, cases originating in the mediastinum are extremely rare. This report discusses an unusual case of mediastinal LAM in a male patient with no abnormal clinical symptoms. The patient, a 70-year-old man, the chest computed tomography (CT) scan revealed an irregular hypodense mass in the left side of the anterior superior mediastinum. Interestingly, the mass did not exhibit significant enhancement in the arterial phase. Instead, it showed striated enhancement in the central area during the venous phase, with no abnormalities observed in the marginal area. To further understand this condition, we conducted a comprehensive review of relevant literature, focusing on the imaging characteristics of mediastinal LAM and the pathogenesis and therapeutic prognosis of LAM. By sharing this information, we aim to enhance understanding and knowledge of this disease.

摘要

淋巴管平滑肌瘤病(LAM)是一种罕见疾病,主要影响育龄期女性。其特征为平滑肌样细胞异常生长。虽然LAM通常发生于肺部,但也可出现在腹膜后和盆腔。然而,起源于纵隔的病例极为罕见。本报告讨论了一例男性纵隔LAM罕见病例,该患者无异常临床症状。患者为一名70岁男性,胸部计算机断层扫描(CT)显示前上纵隔左侧有一不规则低密度肿块。有趣的是,该肿块在动脉期未显示明显强化。相反,在静脉期中央区域呈条纹状强化,边缘区域未见异常。为进一步了解这种情况,我们对相关文献进行了全面回顾,重点关注纵隔LAM的影像学特征以及LAM的发病机制和治疗预后。通过分享这些信息,我们旨在增进对该疾病的理解和认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce53/12455080/65efb546be82/fonc-15-1588165-g001.jpg

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