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胸腔镜楔形切除术治疗源自肺部的低级别纤维黏液样肉瘤(埃文斯肿瘤)伴大量钙化:一个位于意外部位的罕见病例。

Thoracoscopic Wedge Resection for Low-Grade Fibromyxoid Sarcoma (Evans Tumor) with Massive Calcification and Originating from the Lung: A Rare Case in an Unexpected Location.

机构信息

Department of Thoracic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

出版信息

Ann Thorac Cardiovasc Surg. 2024;30(1). doi: 10.5761/atcs.cr.23-00170.

Abstract

We encountered a rare case of low-grade fibromyxoid sarcoma, which is generally known as Evans tumor, with massive calcification originating from the lung. The patient was a 22-year-old man with Duchenne muscular dystrophy who was referred for a detailed investigation of an intrathoracic tumor with massive calcification. Although our preoperative diagnosis was a solitary fibrous tumor originating from the mediastinum or diaphragm, intraoperative thoracoscopy revealed the tumor arising from the left lower lobe without adhesion to the other organs. Considering his medical history, we aimed to preserve lung function and chose wedge resection, which completely removed the tumor. Based on the pathological findings, the tumor was diagnosed as low-grade fibromyxoid sarcoma with massive calcification originating from the lung. Although extremely rare, this tumor should be considered as a differential diagnosis for a solitary lung mass with massive calcification in young adults.

摘要

我们遇到了一例罕见的低度纤维黏液样肉瘤,通常被称为 Evans 肿瘤,其起源于肺部的巨大钙化灶。患者是一名 22 岁的男性,患有杜氏肌营养不良症,因胸腔内巨大钙化灶的详细检查而就诊。尽管我们的术前诊断是源自纵隔或膈肌的孤立性纤维瘤,但术中胸腔镜检查显示肿瘤起源于左下叶,与其他器官无粘连。考虑到他的病史,我们旨在保留肺功能并选择楔形切除术,从而完全切除了肿瘤。根据病理发现,该肿瘤被诊断为起源于肺部的伴有巨大钙化的低度纤维黏液样肉瘤。虽然极其罕见,但对于年轻人的肺部单发巨大钙化肿块,应考虑将其作为鉴别诊断之一。

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