Abstract

Background: Congenital peribronchial myofibroblastic tumor is a rare, solid mesenchymal tumor of the neonate, usually associated with non-immune hydrops fetalis. 

Case Report: We present a case of congenital peribronchial myofibroblastic tumor, in whom a right lung mass was detected in intrauterine life. 12 days after delivery by caesarean section, right lobectomy was performed. The tumor was limited to the lung, and was composed of spindle cells, proliferating around a bronchial unit. Central necrosis and 4-5 mitoses per 10 high power fields were present. The patient is well 26 months after surgery. 

Conclusion: We report this rare tumor with clinical, radiological and pathologic findings and a review of the literature.