Pulmonary Myofibroblastic Tumour Involving the Pericardium and Left Atrium in an 18 Month Infant.

Abstract

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.