title: 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.
authors: Departmant of Pediatric Surgery, Hospital São João, Porto, Portugal.
authors: Ruben
authors: R
authors: Lamas-Pinheiro
authors: Gregory
authors: G
authors: Rodesch
authors: Christine
authors: C
authors: Devalck
authors: Valérie
authors: V
authors: Segers
authors: Karim
authors: K
authors: Khelif
authors: Matteo
authors: M
authors: Cappello
authors: Henri
authors: H
authors: Steyaert
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D005260:Female; D006325:Heart Atria / Q000473:pathology* / Q000601:surgery; D006801:Humans; D033162:Incidental Findings; D007223:Infant; D008175:Lung Neoplasms / Q000473:pathology* / Q000628:therapy; D058628:Myofibroblasts / Q000473:pathology*; D020360:Neoadjuvant Therapy; D009361:Neoplasm Invasiveness; D010496:Pericardium / Q000473:pathology* / Q000601:surgery; D011013:Pneumonectomy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones