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一例肺炎症性肌纤维母细胞瘤的诊断困境

A diagnostic dilemma in a case of pulmonary inflammatory myofibroblastic tumour.

作者信息

Asciak Rachelle, Gouder Caroline, Bilocca David, Montefort Stephen

机构信息

Department of Respiratory Medicine, Mater Dei Hospital, Triq Dun Karm Psaila, Tal-Qroqq MSD 2090, Malta.

出版信息

BMJ Case Rep. 2016 Apr 18;2016:10.1136/bcr-2016-215168. doi: 10.1136/bcr-2016-215168.

Abstract

An 18-year-old man presented to the local hospital in Malta, with dyspnoea, cough, mild haemoptysis, chest pain and night sweats. CT revealed a right hilar mass. Pleural tap, bronchoscopy and open lung biopsy were inconclusive. Biopsies obtained at repeat bronchoscopy and endobronchial ultrasound (EBUS) revealed a likely diagnosis of inflammatory myofibroblastic tumour (IMT). The patient subsequently underwent right pneumonectomy, and histology revealed the presence of two further nodules apart from the main tumour. Follow-up with positron emission tomography (PET)/CT showed the development of a right basal paracardial lesion due to recurrence and the presence of lymph node, pleural and skeletal disease. Despite radiotherapy to the recurrent nodule and chemotherapy, there was skeletal disease progression. Treatment with an anaplastic lymphoma kinase inhibitor, ceritinib, resulted in very good metabolic response. This case report highlights the importance of keeping IMT in mind when the diagnosis of lung tumours is difficult, as delayed diagnosis may lead to worsened prognosis.

摘要

一名18岁男性患者前往马耳他当地医院就诊,出现呼吸困难、咳嗽、轻度咯血、胸痛和盗汗症状。CT显示右肺门肿块。胸腔穿刺、支气管镜检查和开胸肺活检均未得出明确结论。在重复支气管镜检查和支气管内超声(EBUS)时获取的活检结果显示可能诊断为炎性肌纤维母细胞瘤(IMT)。该患者随后接受了右肺切除术,组织学检查发现除主要肿瘤外还存在另外两个结节。正电子发射断层扫描(PET)/CT随访显示因复发出现右心底部心包旁病变以及存在淋巴结、胸膜和骨骼病变。尽管对复发结节进行了放疗和化疗,但骨骼病变仍有进展。使用间变性淋巴瘤激酶抑制剂色瑞替尼进行治疗产生了非常好的代谢反应。本病例报告强调了在难以诊断肺部肿瘤时考虑IMT的重要性,因为延迟诊断可能导致预后恶化。

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本文引用的文献

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