Inflammatory myofibroblastic tumor in children

Abstract

Introduction. Inflammatory myofibroblastic tumor (IMT) is a rare volumetric neoplasm in the childhood which originates from the mesodermal tissue , and by the classification of soft tissue tumors refers to the tumors with intermediate biological potential.Purpose. To present a case of rare volumetric formation in the lungs and mediastinum in children.Material and methods. The publication presents three clinical observations of children with IMT. In all three boys (aged 6, 8 and 15), tumors were localized in the right hemithorax: lower lobe of the lung (two children), upper anterior mediastinum (one) without a reliable organ accessory.Results. There were no specific clinical symptoms of the disease. In two children, the volumetric formation was found accidentally: in one child, a rounded tumor above the liver was found during an ultrasound examination of the abdomen; in the other one, during chest X-ray prophylactic examination. Only in one case, the lung tumor caused respiratory failure during exercise due to the complete atelectasis of the lower lobe and emphysema of the middle lobe which was caused by the growth of the tumor from the parenchyma of the lower lobe into the lumen of the lower lobe and intermediate bronchi. There were no significant shifts in clinical and biochemical blood tests. Cancer markers were negative. Surgery was indicated because of the volume formation topography which was confirmed by the multispiral computed tomography with vascular contrast and fibrobronchoscopy. Surgical volume – thoracotomy, lobtumorectomy (1 child); thoracotomy, pulmonotomy, tumorectomy (1 child); thoracoscopy, removal of the mediastinal tumor (1 child). The most technically difficult was mobilization and removal of the mediastinal tumor when it was separated from the superior vena cava and in the chest aperture where it was intimately fused with the brachiocephalic vein. The postoperative period was without complications. All children recovered.Conclusion. IMT of the thoracic organs is not a rare case. It is not possible to reliably verify the nature of the tumor process in the preoperative period. Surgical treatment is radical and, in some cases, endovideotechnologies may be applied.