Locally invasive pulmonary inflammatory myofibroblastic tumors in children

Abstract

Sir, We read with interest the recent report on pulmonary inflammatory myofibroblastic tumors[1] and congratulate the authors for the successful outcome. We would like to share our experience of a similar case highlighting the invasive and progressive nature of this lesion which can be life threatening. An 8-year-old boy presented with a history of intermittent low-grade fever and cough with expectoration for the past three years. A plain radiograph of the chest showed an opacified mass in the right lower chest. His hematological and biochemical parameters were normal. Contrast enhanced computed tomography (CECT) showed a 4.2×5.5×3.5 cm hypodense mass in the right lower lobe with dense central calcification with normal mediastinal vascular structures [Figure 1]. A CT-guided fine needle aspiration cytology was suggestive of a spindle cell tumor. At thoracotomy, a bony hard white mass located in between and diffusely infiltrating the parenchyma of the middle and lower lobe was seen and we proceeded with lobectomy. It was however densely adherent and infiltrating the pericardium. During dissection of this part, there was massive bleeding. After vascular control and resuscitation, the mass was excised completely [Figure 2] and a small rent in the right atrium closed. He received three units of blood and was transferred to the intensive care unit and put on ventilator. However, he developed bradycardia a few hours later followed by cardiac arrest and could not be revived. The histopathology confirmed inflammatory myofibroblastic tumor. Figure 1 Contrast enhanced computed tomography (CECT) scan of the thorax showing calcified lesion right lung parenchyma abutting the pericardium Figure 2 Calcified mass diffusely infiltrating lung parenchyma with area of intracardiac extension seen superiorly Locoregional including vascular extension is known to occur in 20% cases, mostly in boys around 10 years of age and indicates the aggressive nature of the lesion.[2 3] Berman et al. describe a similar tumor in a nine-year-old boy who initially underwent a thoracotomy and only biopsy followed by echocardiography which confirmed clinical suspicion of invasion of the left atrium.[4] The tumor was later excised through a median sternotomy with cardiopulmonary bypass along with left pneumonectomy and reconstruction of the left atrium. Although complete surgical resection is well accepted as the best modality of management, the progressive and invasive nature of the disease should be kept in mind. Lesions close to the pericardium should undergo echocardiography and the surgeon should be well prepared so that mishaps as happened in our case do not recur.