Pediatric Pulmonology 42:847–850 (2007) Inflammatory Myofibroblastic Tumor of the Trachea Sindhu Sivanandan, MBBS, 1 Rakesh Lodha, MD, 1 * Sandeep Agarwala, MCh, 2 MC Sharma, MD, 3 and Sushil K Kabra, MD 1 Summary. Inflammatory myofibroblastic tumor of the trachea is a rare benign tumor in children. We describe a 9-year-old girl who presented with recurrent episodes of wheeze and severe respiratory distress requiring mechanical ventilation. She had recurrent collapses of the right lung and a chest CT and bronchoscopy confirmed the presence of an obstructing mass lesion at the carina. The lesion, 1.5 cm  1.5 cm in size, was debulked by rigid bronchoscopyand histopathology revealed features of inflammatory myofibroblastic tumor. A repeat bronchoscopy at 1 month of follow up did not reveal any residual lesion. Pediatr Pulmonol. 2007; 42:847–850. ß 2007 Wiley-Liss, Inc. Key words: inflammatory myofibroblastic tumor of the trachea; tracheal tumor. INTRODUCTION Inflammatory myofibroblastic tumor/pseudotumor re- presents a group of unusual pseudoneoplastic lesions prototypically found in the lung. Similar lesions are also observed in a variety of extrapulmonary sites. 1 Although inflammatory myofibroblastic tumor is a common lesion in children, there are very few published reports of such an entity in the trachea. 2 We report here a child with inflammatory myofibroblastic tumor of the trachea. CASE REPORT A 9-year-old girl presented to the emergency with severe respiratory distress, cyanosis and wheeze for 12 hr. Examination revealed an emaciated girl, with weight 16 kg. She was in altered sensorium and had severe chest retractions and audible wheeze. Pulse rate was 120/min, respiratory rate 72/min and saturation on oxygen by face mask was 88%. Air entry was decreased in entire right hemithorax with dull percussion note. She did not improve on nebulized bronchodilators and intravenous terbutaline and required intubation and mechanical ventilation. Detailed history revealed that she had been symptomatic for the past 2 months with cough, fast breathing, recurrent episodes of wheeze and respiratory distress, and had required mechanical ventilation for 7 days, 2 weeks prior to this episode. She was on inhaled bronchodilators and steroids. Initial working diagnosis was acute severe asthma and right lung collapse possibly due mucous plug or foreign body aspiration. Chest X-ray at admission (Fig. 1) showed collapse of the right lung. During the initial 96 hr of mechanical ventilation she had recurrent collapses of the right lung that improved spontaneously and had bloody endotracheal aspirates. At this point, a possibility of a foreign body or a right bronchial mass was strongly considered. A diagnostic fiber-optic bronchoscopy was done that revealed a sessile 1.5 cm mass in the trachea near the carina extending up to the right main bronchus. The above finding was confirmed by a CT scan of the chest (Fig. 2). Subsequently the mass was removed endoscopically with forceps. A thorough debulking was ensured. Microscopic examination of the lesion showed squamous lining epithelium and a spindle cell lesion in the sub-epithelial region. The spindle cells were arranged in fascicles and had scant to moderate amount of cytoplasm and indistinct cell outlines. Pleomorphism was minimal and occasional mitosis was seen. There was sprinkling of lymphocytes amidst the spindle 1 Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. 2 Department of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. 3 Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. *Correspondence to: Rakesh Lodha, Assistant Professor, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India E-mail: rakesh_lodha@hotmail.com Received 12 December 2006; Revised 24 April 2007; Accepted 24 April 2007. DOI 10.1002/ppul.20651 Published online 10 July 2007 in Wiley InterScience (www.interscience.wiley.com). ß 2007 Wiley-Liss, Inc.