A case of neurofibroma adjacent to lateral thoracic meningocele with massive intrathoracic hemorrhage due to tumor rupture

Abstract

A 61-year-old man with neurofibromatosis type 1 (NF1) presented with sudden onset of left-sided chest pain. Contrast-enhanced computed tomography (CT) showed active bleeding from a left posterior mediastinal tumor adjacent to a lateral thoracic meningocele, accompanied by massive left hemothorax. The patient was in shock and urgently transferred to our hospital, where emergency thoracotomy with evacuation of the hemothorax and resection of the mediastinal tumor were performed. Due to the indistinct boundary between the tumor and dura mater, partial dural resection and reconstruction with artificial dura mater were required. Postoperatively, cerebrospinal fluid leakage and symptoms of intracranial hypotension persisted, necessitating spinal drainage and resuturing of the dural defect on postoperative day 10.

NF1 is typically characterized by cutaneous, skeletal, and neural manifestations, but spontaneous hemothorax is a rare complication. The bleeding source is often a thoracic or tumor vessel, and treatment may involve surgery or interventional radiology. In cases where the tumor is adjacent to a lateral thoracic meningocele, as in the present case, careful management of the dura mater is essential, and multidisciplinary collaboration is crucial for optimal treatment.