Mediastinal and pulmonary infantile myofibromatosis: an unusual surgical presentation.

A 4-week-old boy was extensively investigated for stridor and respiratory distress and was found to have a soft tissue mass superior to the left hilum and emphysema of the entire left lung. An exploratory thoracotomy was undertaken for diagnosis and possibly to improve respiratory distress. Intraoperatively, a firm plaquelike mass was identified encasing the entire hilum including left pulmonary artery and left main bronchus. It became apparent that a left pneumonectomy was needed to be performed to resect the tumor completely and achieve hemostasis. Histopathologic examination revealed infantile myofibromatosis with multiple foci within the entire lung parenchyma as well as in the hilar mass. The child is completely recurrence-free and symptom-free after 6 years of follow-up. The literature review was carried out to discuss management of this rare but benign and surgically challenging condition.