Successful surgical treatment of massive spontaneous hemothorax due to intrathoracic secondary degeneration of a neurofibroma from mediastinal involvement of type 1 neurofibromatosis

摘要

Massive hemothorax caused by a mediastinal mass is extremely rare. Herein, we present a case of successful surgery for a massive hemothorax caused by intrathoracic cystic hemorrhagic degeneration of a neurofibroma in a patient with neurofibromatosis type 1 (NF1). A 44-year-old man with NF1 was admitted to our emergency department for chest pain and dyspnea. Image studies revealed a massive hemothorax caused by a posterior mediastinal mass adjacent to the descending aorta and the spinal canal. The tumor, located at the fourth to the sixth thoracic vertebra, had diffusely and extensively infiltrated around proliferative vessels. It was successfully excised via thoracostomy after aortic cross-clamping preparation. Final pathology revealed that the tumor was cystic hemorrhagic degeneration of a neurofibroma from mediastinal involvement of NF1. The patient had an uneventful postoperative course and was discharged on the 10th postoperative day. There was no recurrence of tumor or hemothorax six months after surgery.