Inflammatory myofibroblastic tumours of the respiratory tract: paediatric case series with varying clinical presentations

Abstract

Inflammatory myofibroblastic tumour are a rare but clinically important and pathologically distinct lesion of the respiratory tract in children. The cases in this series highlight some of the varied clinical presentations of inflammatory myofibroblastic tumours, and illustrate some of this tumour's different anatomical locations within the paediatric respiratory tract.

Pulmonary Inflammatory Myofibroblastic Tumor in Children: A Case Report and Brief Review of Literature

“…Bronchoscopy is usually performed when there exists a suspect of endoluminal lesion. It is a useful diagnostic and therapeutic tool since it has both a function of removing tumoral obstruction and providing biopsy samples ( 13 , 19 ).…”

A rare aetiology of respiratory failure in a 10-year-old boy: inflammatory myofibroblastic tumour

“…In rare cases, patients have been reported to have arthalgia and clubbing of the fingers which got resolved after resection of the tumour 4. However, patients might also be asymptomatic and the tumour may be found incidentally on imaging or direct visualisation through bronchoscopy 4. Our patient presented with symptoms of non-resolving cough and severe respiratory distress and was found to have complete atelectasis of the left lung which has been rarely reported before 10 12…”

“…In rare cases, patients have been reported to have arthalgia and clubbing of the fingers which got resolved after resection of the tumour 4. However, patients might also be asymptomatic and the tumour may be found incidentally on imaging or direct visualisation through bronchoscopy 4.…”

“…It has been reported that transthoracic or transbronchial needle biopsy may not be definitive and it may be difficult to differentiate between IMT and other neoplasms including fibrohistiocytic neoplasms, nodular sclerosing Hodgkin lymphoma, primary lung cancer and mediastinal fibrosis 2. Therapeutically, complete surgical resection, has shown to have excellent prognosis and low rate of recurrence 3 4 10 13. In cases where resection is not possible, use of lasers, steroids and non-steroidal anti-inflammatory drugs have shown some promise 15 16.…”

Lesão Tumoral Primária do Pulmão em Adolescente

“…7 A apresentação clínica é muito variável consoante a localização da lesão: os TMIP centrais são habitualmente diagnosticados mais cedo por obstrução da traqueia, carina ou brônquio principal, enquanto os TMIP periféricos se apresentam com sintomas inespecíficos, como tosse, febre, dispneia, anemia, perda ponderal, dor torácica e hemoptises, que condicionam um diagnóstico mais tardio. [4][5][6][7][8][9] A radiografia de tórax demonstra habitualmente uma lesão nodular única, periférica, de contornos regulares e diâmetro variável (1,2 a 15 cm), com predileção pelos lobos inferiores. Mais raramente apresenta-se sob a forma de lesões múltiplas ou como uma massa endobrônquica, estando indicada a investigação por broncoscopia.…”