Inflammatory myofibroblastic lung tumor with dysphagia in a 4-year-old child

Abstract

Inflammatory myofibroblastic tumors of the lung are rare in adults but common among pediatric lung tumors. A 4-year-old girl was evaluated for respiratory symptoms, dysphagia, and weight loss. Radiology revealed a right hilar mass that persisted despite antibiotic treatment. On exploration, a hilar mass involving the pulmonary vasculature, diaphragm and gastroesophageal junction was found. Aggressive surgical excision including a right pneumonectomy was performed. Histopathology confirmed an inflammatory myofibroblastic tumor with no features of malignancy. At the 2-year follow-up, the child was clinically stable with no recurrence. Dysphagia is a very rare presentation of such tumors.

Thoracic Multidetector Computed Tomography Evaluation of Inflammatory Myofibroblastic Tumor of the Lung in Pediatric Patients in the Era of Modern Diagnosis

“…Second, previously published case reports and studies with smaller patient populations described various locations of IMT of the lung that were inconsistent with each other, and also without recognition that the vast majority of pediatric IMT of the lung are pleural-based, as seen in our study. [4][5][6][7]17,18,[21][22][23][24] The pattern of contrast enhancement of pediatric IMT of the lung in our study is also another new finding that is different from the previously published studies. In contrast to the finding of various patterns of contrast enhancement (ie, nonenhancing, homogeneously enhancing, and heterogeneously enhancing) described in previously published studies obtained with a single-detector CT, all IMT of the lung masses showed heterogeneous contrast enhancement in our study with MDCT.…”

“…This sets our study apart from many of the previously published studies on pediatric IMT and may explain some of the differences in imaging findings. In addition, the results of our study, which focused on thoracic MDCT findings, with the largest pediatric patient population at the present time, provide new, more specific information on the characteristic MDCT attributes of IMT of the lung in children, many of which differ from the findings of previously published studies obtained with either single-detector CT, mixed pediatric and adult population, or overall smaller patient population size 4–7,17,18,21–24,31,32…”

“…In addition, the results of our study, which focused on thoracic MDCT findings, with the largest pediatric patient population at the present time, provide new, more specific information on the characteristic MDCT attributes of IMT of the lung in children, many of which differ from the findings of previously published studies obtained with either single-detector CT, mixed pediatric and adult population, or overall smaller patient population size. [4][5][6][7]17,18,[21][22][23][24]31,32 First, a previously published study by Agrons et al 6 consisting of both pediatric and adult patients stated that IMT of the lung has an anatomic bias for the lower lobes. In contrast, the results of our study show that there is no significant lobar preference in pediatric patients with IMT of the lung.…”

“…15,16 Currently, published descriptions of the imaging findings of IMT of the lung in children have been limited to case reports and small studies. 4,5,[11][12][13][17][18][19][20][21][22][23][24] Furthermore, to our knowledge, no large study specifically investigating the characteristic thoracic MDCT features of IMT of the lung has been published since the advent of MDCT, which can provide improved characterization of lung masses with high temporal and spatial resolution and contrast enhancement pattern. Therefore, the purpose of this study is to investigate the characteristic thoracic MDCT findings of pathologically proven IMT of the lung in children in the era of modern understanding based on refined pathologic diagnosis.…”

“…In affected pediatric patients with IMT of the lung, thoracic multidetector computed tomography (MDCT) is frequently obtained for a more precise diagnostic imaging evaluation after chest radiography, which is often limited and nonspecific in the characterization of thoracic masses 15,16. Currently, published descriptions of the imaging findings of IMT of the lung in children have been limited to case reports and small studies 4,5,11–13,17–24. Furthermore, to our knowledge, no large study specifically investigating the characteristic thoracic MDCT features of IMT of the lung has been published since the advent of MDCT, which can provide improved characterization of lung masses with high temporal and spatial resolution and contrast enhancement pattern.…”