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中央气道炎性肌纤维母细胞瘤:治疗与分子分析

Inflammatory myofibroblastic tumour of the central airways: treatment and molecular analysis.

作者信息

Iyer Aparna, Radonic Teodora, Heukamp Lukas C, Thunnissen Erik, Daniels Johannes M A

机构信息

Dept of Pulmonary Medicine, Amsterdam University Medical Centre, Amsterdam, The Netherlands.

Dept of Pathology, Amsterdam University Medical Centre, Amsterdam, The Netherlands.

出版信息

ERJ Open Res. 2021 Mar 22;7(1). doi: 10.1183/23120541.00151-2020. eCollection 2021 Jan.

Abstract

Inflammatory myofibroblastic tumours (IMT) are a rare cause of endobronchial masses in adults. Surgery has been the mainstay of treatment of endobronchial IMTs, based on the potential for recurrence. Interventional pulmonology has emerged as a minimally invasive and lung function preserving modality in management of airway obstruction due to tumours. We present a series of three adult patients with IMT treated endobronchially with a short discussion on its potential role. We also discuss how molecular analysis of IMTs for mutations in genes such as and might provide insights into clinical behaviour and potential targetable therapy in advanced, unresectable and metastatic cases.

摘要

炎性肌成纤维细胞瘤(IMT)是成人支气管内肿物的罕见病因。基于复发的可能性,手术一直是支气管内IMT的主要治疗方法。介入肺脏病学已成为一种微创且能保留肺功能的治疗因肿瘤导致气道阻塞的方式。我们报告了3例接受支气管内治疗的成年IMT患者,并简要讨论了其潜在作用。我们还讨论了对IMT进行诸如 和 等基因突变的分子分析如何能为晚期、不可切除和转移性病例的临床行为及潜在的靶向治疗提供见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cfb2/7983254/b77dbce1288b/00151-2020.01.jpg

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