耳鼻喉科炎症性肌成纤维细胞瘤

Lucie Zeinerová, M. Černý, J. Laco, J. Dědková, V. Chrobok
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摘要

炎性肌成纤维细胞瘤(IMT)是一种罕见的边缘性生物行为肿瘤,由成纤维细胞和肌成纤维细胞组成,并伴有非肿瘤性炎症细胞。它主要发生在儿童和年轻人身上。本病最常见于腹部软组织(网膜、肠系膜等)、肺部,也有胃肠道其他部位和其他器官的报道。在头颈部IMT并不常见,它可能发生在喉部、气管或鼻窦。主要的治疗方法是完全的手术切除。放射,化疗或生物治疗方式用于侵袭性,不可切除,复发性肿瘤或转移的情况。目的:通过对一小部分患者的病例报告和最新的文献信息,作者希望分享耳鼻喉科IMT的罕见诊断,其可能的临床表现,鉴别诊断和适当治疗的知识。患者组,病例报告:本文介绍了2013-2020年在赫拉德茨克拉洛韦大学医院耳鼻喉科和头颈外科接受IMT治疗的患者。病例11岁男孩的进展性呼吸困难引起的息肉状气管肿瘤的讨论更详细。在支气管镜辅助下,经外部手术入路切除肿瘤。组织学检查证实为IMT。结论:IMT是一种罕见的肿瘤,必须纳入呼吸道肿瘤的鉴别诊断,特别是在儿童和青少年中。由于有局部复发的危险,治疗包括完全手术切除和定期随访。关键词:炎性肌成纤维细胞瘤-儿童呼吸困难-气管肿瘤
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Inflammatory myofibroblastic tumour in otorhinolaryngology
Introduction: Inflammatory myofibroblastic tumour (IMT) is a rare tumour with borderline biological behaviour composed of fibroblasts and myofibroblasts and accompanied by non-neoplastic inflammatory cells. It occurs mostly in children and young adults. The disease is most commonly seen in abdominal soft tissues (omentum, mesentery, etc.), in the lungs and also has been reported in other parts of gastrointestinal tract and other organs. In the head and neck region IMT is not so common, it may occur in larynx, trachea or nasal sinuses. The primary therapeutic approach is a complete surgical excision. Radiation, chemotherapy or biological treatment modalities are used in aggressive, non-resectable, and recurrent tumours or in case of metastases. Aims: Through a case report on a small set of patients and up-to-date information from the literature, the authors want to share knowledge about the rare diagnosis of IMT in ENT, its possible clinical manifestations, differential diagnosis and appropriate treatment. The group of patients, the case report: The paper presents patients with IMT treated at the Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Hradec Kralove in the years 2013–2020. The case of a 11-year-old boy with progressive dyspnoea caused by polypoid tracheal tumour is discussed in more detail. The tumour was removed by external surgical approach with bronchoscopy assistance. Histological examination proved an IMT. Conclusion: IMT is a rare neoplasm which must be included in the differential diagnosis of tumours of the respiratory tract, especially in children and young adults. The treatment consists of complete surgical removal and regular follow-up due to the risk of local recurrence. Keywords: inflammatory myofibroblastic tumour – dyspnoea in a child – tracheal tumour
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