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Pulmonary myofibroblastic tumour involving the pericardium and left atrium in an 18 month infant

par Lamas-Pinheiro, Ruben;Rodesch, Grégory ;Devalck, Christine ;Segers, Valérie ;Khelif, Karim ;Cappello, Matteo ;Steyaert, Henri
Référence Annals of thoracic and cardiovascular surgery, 22, 5, page (312-314)
Publication Publié, 2016

Article révisé par les pairs

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Résumé :

Inflammatory myofibroblastic tumor (IMT) is the most frequent primary lung tumor in children and it may be locally aggressive. The management of a locally advanced pulmonary IMT in an 18 month-old female child is presented. A left pulmonary mass was incidentally found on the computerized tomography (CT) scan of a child with persistent systemic inflammatory syndrome. Biopsy confirmed the diagnosis; after preoperative corticotherapy,left pneumonectomy was performed. The pericardium and left atrium were invaded and resected,requiring pericardial reconstruction. There is no relapse at four years of follow-up. Steroids play a role in tumor size reduction,but marginal resection is the gold standard. Extended approaches are feasible and often required in advanced cases.