| Summary: | Congenital peribronchial myofibroblastic tumor (CPMT) is a rare entity recognized in the WHO classification of pulmonary neoplasms. According to available literature, it is a benign tumor with a high mortality rate exceeding 50%. It is partially attributed to polyhydramnios, hydrops, prematurity, respiratory distress or adverse surgical outcomes due to intraoperative bleeding. Herein we present a case of congenital peribronchial myofibroblastic tumor in a premature male infant who was born at 31 weeks gestation due to polyhydramnios and premature rupture of membranes. Soon after birth, he required intubation due to worsening respiratory distress. Imaging demonstrated a large right chest mass causing mediastinal shift. Surgical intervention was attempted, which was challenging due to intraoperative bleeding and tumor retraction. The patient expired soon after the surgery. Hence, in this report we would like to share our experience with this difficult diagnosis and treatment of this rare tumor.
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