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朗格汉斯细胞组织细胞增多症累及胸腺和心脏并伴有胸腺瘤:一例报告

Langerhans Cell Histiocytosis Involving the Thymus and Heart With Simultaneous Thymoma: A Case Report.

作者信息

Ji Ting, Zhong Yuxia, Cheng Deyun

机构信息

Department of Respiratory and Critical Care Medicine, West China Hospital of Sichuan University, Sichuan, China.

出版信息

Front Oncol. 2022 Apr 25;12:890308. doi: 10.3389/fonc.2022.890308. eCollection 2022.

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal expansion of CD1a+/CD207+ cells in lesions. The most frequent sites involved are bone and, less commonly, lymph nodes, lungs, and skin. The thymus or heart is rarely involved with LCH. In this case, we present a 73-year-old woman with a mediastinal mass. Histopathology after thymectomy identified this mass as type AB thymoma; notably, subsequent immunohistochemical tests showed lesions of LCH scattered in the region of thymoma. 18-Fluorodeoxyglucose PET/CT (18-FDG-PET/CT) was performed to make an overall assessment of the extent of this disease, which demonstrated suspicious cardiac involvement of LCH. This report highlights the importance of differentiating abnormalities of the thymus or mediastinal mass from LCH and the necessity of comprehensive evaluation for patients with LCH.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,其特征为病变中CD1a+/CD207+细胞的克隆性扩增。最常受累的部位是骨骼,较少见的是淋巴结、肺和皮肤。胸腺或心脏很少受累于LCH。在本病例中,我们报告一名73岁女性,患有纵隔肿块。胸腺切除术后的组织病理学检查将该肿块确定为AB型胸腺瘤;值得注意的是,随后的免疫组化检测显示LCH病变散在于胸腺瘤区域。进行了18-氟脱氧葡萄糖PET/CT(18-FDG-PET/CT)检查以全面评估该疾病的范围,结果显示LCH可疑累及心脏。本报告强调了将胸腺或纵隔肿块异常与LCH进行鉴别诊断的重要性以及对LCH患者进行综合评估的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/16ff/9082352/ffeee7726729/fonc-12-890308-g001.jpg

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