Paediatric airway inflammatory myofibroblastic tumours: a decade of lessons from a quaternary centre

Inflammatory myofibroblastic tumours (IMTs) are rare low-grade neoplasms that can occur in the airway and can result in airway compromise and respiratory distress. There are no clear guidelines on the management of airway IMT. This retrospective study discusses the management and outcomes of patients presenting with an airway IMT at our centre.

Five patients diagnosed with an IMT in the larynx, trachea or bronchi between January 2014 and November 2024 were included. Initial presentation, initial and definitive management, complications, surveillance method and recurrence rates were recorded.

The initial treatment was endoluminal debulking for histological diagnosis. Immunohistochemistry was positive for anaplastic lymphoma kinase (ALK) in four patients. The upfront definitive treatment was open resection in two patients and endoscopic removal in one patient. Both patients with upfront non-surgical treatment failed, requiring open surgical resection. One patient was initially treated with an ALK-inhibitor and one with systemic steroids and nonsteroidal anti-inflammatory agents. Tumour surveillance was via microlaryngobronchoscopy and cross-sectional imaging. There was no tumour recurrence in the surgically treated group despite tumour present at the margins. There were no deaths in this series.

Airway lesions such as IMT are rare. Our management includes a multidisciplinary approach to treatment, favouring upfront surgery tailored to the tumour location and characteristics.