Endobronchial Malignant Peripheral Nerve Sheath Tumor in a Pediatric Patient.

A 13-year-old girl presented to the emergency department with exertional dyspnea. She had no notable medical history. A chest radiograph revealed left pulmonary atelectasis. Bronchoscopy showed an endobronchial tumor obstructing the left main bronchus, protruding into the trachea. Endobronchial polypectomy was performed under general anesthesia. The specimen was found to be a malignant peripheral nerve sheath tumor. The tumor had a local recurrence, however it probably went into spontaneous regression. She is alive 25 years after the initial surgery. This is the first reported case of an endobronchial malignant peripheral nerve sheath tumor in a pediatric patient.