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气管内炎性肌纤维母细胞瘤:成人中央气道阻塞的罕见原因。

Endotracheal inflammatory myofibroblastic tumour: A rare cause of central airway occlusion in adults.

作者信息

Lourdesamy Anthony Albert Iruthiaraj, Satnam Singh Taranjit Kaur, Ng Khai Lip, Abdul Rahaman Jamalul Azizi

机构信息

Respiratory Unit Hospital Taiping Taiping Malaysia.

Department of Radiology Hospital Taiping Taiping Malaysia.

出版信息

Respirol Case Rep. 2022 Jun 7;10(7):e0984. doi: 10.1002/rcr2.984. eCollection 2022 Jul.

Abstract

Inflammatory myofibroblastic tumour (IMT) is an unusual myofibroblastic spindle cell neoplasm that is rarely discovered in the airway of adults. Previously, it was regarded as a reactive lesion and was infamously known as inflammatory pseudotumour before recent insights revealed that significant majority of cases harboured neoplastic genes. Diagnosis is difficult as clinical presentation and imaging findings are non-specific. Diagnosis and a favourable prognosis require the complete resection of the tumour. Detection of the anaplastic lymphoma kinase expression via immunohistochemistry expedites diagnosis. We report a young adult with an endotracheal mass occluding the central airway. The patient successfully underwent bronchoscopic resection using interventional techniques. IMT was diagnosed. No recurrence was seen after a year of surveillance. Due to the rarity of the disease, the recurrence rates for large airway disease is unknown. Recurrence rates for pulmonary lesions is reported to be lower compared to extrapulmonary IMTs and recurrence is unlikely if compete surgical excision is achieved.

摘要

炎性肌纤维母细胞瘤(IMT)是一种不常见的肌纤维母细胞性梭形细胞瘤,在成人气道中很少见。以前,它被视为一种反应性病变,在最近的研究揭示大多数病例存在肿瘤基因之前,它以炎性假瘤而闻名。由于临床表现和影像学表现不具有特异性,诊断较为困难。诊断和良好的预后需要完整切除肿瘤。通过免疫组织化学检测间变性淋巴瘤激酶表达可加快诊断。我们报告了一名年轻成人,其气管内肿物阻塞了中央气道。该患者成功地通过介入技术进行了支气管镜切除。诊断为IMT。经过一年的随访未见复发。由于该疾病罕见,大气道疾病的复发率尚不清楚。据报道,肺部病变的复发率低于肺外IMT,如果实现完整的手术切除,则不太可能复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fb55/9174595/0aa7da0e9262/RCR2-10-e0984-g002.jpg

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