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伪装成纤维性纵隔炎的滤泡树突状细胞肉瘤。

Follicular dendritic sarcoma masquerading as fibrosing mediastinitis.

作者信息

Cingam Shashank Reddy, Al Shaarani Majd, Takalkar Amol, Peddi Prakash

机构信息

Department of Internal Medicine, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA.

Department of Pathology and Translational Pathobiology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA.

出版信息

BMJ Case Rep. 2017 Jun 13;2017:bcr-2016-218889. doi: 10.1136/bcr-2016-218889.

Abstract

Fibrosing mediastinitis (FM) is a rare disorder resulting from abnormal immunological-mediated fibro-proliferative reaction in the mediastinum. Here, we describe a case of a 46-year-old female with an incidentally found 11×9 cm posterior mediastinal mass. Multiple biopsies of this unresectable, 18-fluorodeoxyglucose avid mass revealed marked fibrosis without any evidence of malignancy, suggesting idiopathic fibrosing mediastinitis as our initial diagnosis. Multiple interventions including a trial of steroids, fluconazole, and azathioprine to target fibrosing mediastinitis were not successful. Repeat biopsy was consistent with primary mediastinal follicular dendritic cell sarcoma. The manuscript highlights the heightened need for suspecting occult malignancies in cases of FM presenting with an indeterminate cause.

摘要

纤维性纵隔炎(FM)是一种罕见的疾病,由纵隔内异常的免疫介导的纤维增生反应引起。在此,我们描述一例46岁女性,偶然发现后纵隔有一个11×9 cm的肿块。对这个无法切除、摄取18氟脱氧葡萄糖的肿块进行多次活检,结果显示有明显纤维化,无任何恶性证据,初步诊断为特发性纤维性纵隔炎。针对纤维性纵隔炎进行的包括类固醇、氟康唑和硫唑嘌呤试验在内的多种干预均未成功。重复活检结果与原发性纵隔滤泡树突状细胞肉瘤一致。该病例强调,对于病因不明的纤维性纵隔炎病例,更需怀疑隐匿性恶性肿瘤。

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